Disorders of thhe Hair and Nails

Question 1

1. The distal nail matrix is visible at the following portion of the nail unit:

A. Onychodermal band
B. Hyponychium
C. Lunula
D. Cuticle
E. Lateral nailfold

Answer 1

Correct choice: C. Lunula

Explanation: The distal nail matrix is located at the lunula, which is visible as the half-moon structure at the base of the nail. The proximal matrix is located under the proximal nailfold. Damage to the proximal nail matrix produces defects at the dorsal nailplate, and damage to the distal nail matrix produces produces defects at the ventral nail plate.
Onychodermal band: A fine, pallid, translucent band at the junction of the distal nail bed and hyponychium.
Hyponychium: The hyponychium is the area of epithelium, particularly the thickened portion, underlying the free edge of the nail plate on the nail.
Cuticle: The cuticle is an area of keratin joining the skin of the posterior nail fold to the nail plate. Loss of cuticle results in paronychia.
Lateral nailfold: The nail plate emerges from the proximal nail fold and is bordered on either side by the lateral nail folds (paronychium). The skin proximal to the nail that covers the nail fold is
the eponychium. The tissue distal to the eponychium in contact with the nail represents the cuticle. The lunula, or lunulae (‘little moon’, is the crescent-shaped whitish area of the bed of a fingernail or toenail. The lunula is the visible part of the root of the nail.

Question 2

2. A 28-year-old male presents with three coin-shaped areas of scalp alopecia without scaling or erythema. Which of the following is most likely to be associated with alopecia areata?

A. Hypothyroidism
B. Seborrheic dermatitis

C. Tinea capitis
D. Congestive heart failure
E. Chronic kidney disease

Answer 2

Correct choice: A. Hypothyroidism

Explanation: Alopecia areata is associated with autoimmune diseases, especially thyroid disease, hypo- or hyperthyroidism. It presents with well-defined patches of alopecia without scale or erythema. Seborrheic dermatitis may result in temporary hair thinning, but does not result in coin- shaped patches of alopecia. Tinea capitis presents with itchy scale on the scalp and possibly temporary poorly defined alopecia. Congestive heart failure and chronic kidney disease are not associated with alopecia areata. Hypothyroidism and thyroid autoimmunity are commonly associated with alopecia aerata (AA). AA cases should be screened for thyroid dysfunction and thyroid autoimmunity even in the absence of clinical manifestations suggestive of these diseases.
Autoimmunity is proposed to play a role in AA. The autoimmune process appears to be T-cell mediated although antibodies to anagen phase hair follicle structures are seen in about 90% of patients. This autoimmune etiology has been proposed also on the basis of its association with various autoimmune diseases, including thyroid disorders, vitiligo, pernicious anemia, diabetes mellitus, lupus erythematosus, myasthenia gravis, lichen planus, atopic dermatitis, autoimmune polyendocrine syndrome type I and celiac disease. Thyroid disorders and vitiligo have the strongest association with AA. Thyroid disorders that may be associated with AA include hypothyroidism, Hashimoto’s thyroiditis, Graves’ disease and simple goiter. Among these, hypothyroidism was the most frequent association.

Question 3

3. Blue lunulae may result from which of the following?

A. Chronic bronchiectasis
B. Hydroxyurea
C. Nail-patella syndrome
D. Renal failure
E. Alopecia areata

Answer 3

Correct choice: B. Hydroxyurea

Explanation: Alteration in the color of lunula can be an indication of either a cutaneous or systemic disorder or a systemic drug side effect. Hydroxyurea, an antitumor systemic agent (a ribonucleoside diphosphate reductase inhibitor) used in the treatment of refractory psoriasis as well as in the variety of neoplastic disorders is known to cause brownish pigmentation of the nails but hydroxyurea induced blue lunula is very rare. It was reported in a 45-year-old man with chronic recalcitrant plaque psoriasis on oral hydroxyurea 500 mg twice daily. Lunular pigmentation in finger and toenails developed two weeks later. During follow up, pigmentation remained localized to the proximal portion of nails. Yellow nails may result from chronic bronchiectasis. Nail-patella syndrome is associated with triangular lunulae. Renal failure may produce absent lunulae, and alopecia areata may result in red lunulae.

Question 4

4. Which of the following is not a feature of Bazex-Dupre-Christol syndrome?

A. peripheral vasodilation with cyanosis
B. multiple basal cell carcinomas
C. hypohidrosis
D. milia
E. epidermoid cysts

Answer 4

Correct choice: A. peripheral vasodilation with cyanosis

Explanation: Peripheral vasodilation with cyanosis is not found in Bazex-Dupre-Christol syndrome; it is a feature of Rombo syndrome. Bazex-Dupre-Christol syndrome includes multiple BCC, follicular atrophoderma, hypohidrosis, milia, epidermoid cysts, and hypotrichosis.

Question 5

5. A 60-year-old woman presents in your dermatology clinic with proximal nail fold erythema and edema, fingertip tenderness and swelling, and eyelid dermatitis. She thinks she may have an allergic contact dermatitis to products in her nail polish. Patch testing with the North American-80 comprehensive series will most likely be positive to which of the following allergens in this patient’s case?

A. Tosylamide formaldehyde
B. Potassium hydroxide
C. Polyester resin
D. Kaolin
E. Butyl acetate

Answer 5

Correct choice: A. Tosylamide formaldehyde

Explanation: Allergic contact dermatitis is seen in persons sensitive to tosylamide formaldehyde, who may develop proximal nail fold erythema and edema, fingertip tenderness and swelling, and/or eyelid dermatitis.
2 - Potassium hydroxide:
Potassium and sodium hydroxide are the main ingredients in cuticle remover, which destroys keratin that forms excess cuticular tissue on the nail plate. The main side effect is irritant contact dermatitis and paronychia, not allergic contact dermatitis.
3 - Polyester resin:
Resins, formaldehyde, acetates, and acrylics are the main ingredients of nail hardeners which increase nail strength and prevent breakage. Its use predisposes to allergic contact dermatitis to formaldehyde, however it would be less common than tosylamide formaldehyde and the clinical presentation would differ.
4 - Kaolin:
Kaolin, pumice and talc are the main ingredients in nail buffing cream which smoothes ridges in nails. There are practically no adverse reactions.
5 - Butyl acetate:
Butyl acetate, acetone, alcohol, and ethyl acetate are the main ingredients in nail enamel removal. Its most common adverse reaction is irritant contact dermatitis, not allergic contact dermatitis.

Question 6

6. Splinter hemorrhage of the nail can be seen with which parasitic infections?

A. Scabies
B. Trichinosis
C. Sparganosis
D. Dracunculiasis
E. Gnathostomiasis

Answer 6

Correct choice: B. Trichinosis

Explanation: Trichinosis can cause splinter hemorrhage of the nails. The other listed infections are not associated with splinter hemorrhage of the nails.
c

Question 7

7. Tricholemmoma is which type of hair follicle tumor?

A. Tumor of the hair germ cells
B. Tumors of matrical differentiation
C. Tumors of the external root sheath
D. Tumor of isthmus differentiation
E. Tumor of the internal root sheath

Answer 7

Correct choice: C. Tumors of the external root sheath

Explanation: Tricholemmoma is a tumor of the external root sheath. Tumors of the hair germ cells include trichoblastoma and trichoepithelioma. Tumors of matrical differentiation include pilomatricoma. Tumors of the external root sheath include pilar cyst, proliferating tricholemmal cyst, tricholemmoma, tricholemmal carcinoma. Tumor of the isthmus differentiation include tumor of the follicular infundibulum.
c

Question 8

8. A 60-year-old patient develops a green-black to green-blue discoloration of one of her toenails. You suspect she may have green nail syndrome. Which of the following is not a predisposing factor to the development of green nail syndrome?

A. excessive use of detergents.
B. excessive use of soaps
C. nail trauma
D. melanocytic nevus
E. frequent and prolonged exposure to water

Answer 8

Correct choice: D. melanocytic nevus

Explanation: In green nail syndrome (chloronychia), the nail develops green–black to green–blue discoloration due to pyocyanin, a blue–green pigment produced by P. aeruginosa. Predisposing factors include frequent or prolonged exposure to water, excessive use of detergents and soaps, nail trauma and other causes of onycholysis. The diagnosis of green nail syndrome is usually clinical; if necessary, it can be confirmed by Gram stain and culture of exudate and nail fragments. The differential diagnosis includes a subungual hematoma, melanocytic nevus, melanoma, and Aspergillus infection.

Question 9

9. Which of the following nutritional deficiencies is associated with perifollicular hemorrhage?

A. Vitamin C
B. Niacin
C. Vitamin B12
D. Folate
E. Zinc

Answer 9

Correct choice: A. Vitamin C

Explanation: Vitamin C deficiency, or scurvy, may lead to perifollicular hemorrhage.
Niacin deficiency may result in a photo-distributed eruption, diarrhea, and psychiatric symptoms (diarrhea, dermatitis, and dementia). Vitamin B12 may lead to glossitis with a shiny, painful tongue. Zinc deficiency may result in a periorificial dermatitis.

Question 10

10. A 73-year-old man presents with a “red streak” involving the nail plate of the first digit. You make the diagnosis of longitudinal erythronychia. What is the most common underlying cause:

A. Melanoma
B. Myxoid cyst
C. Subungual keratoacanthoma
D. Carbon Monoxide toxicity
E. Onychopapilloma
c

Answer 10

Correct choice: E. Onychopapilloma

Explanation: Onychopapilloma represents a benign tumor of the nail unit and is the most common etiology of longitudinal erythronychia. Myxcoid cysts are mucin-filled pseudocysts that may be connected to the joint space and often present as longitudinal grooving of the nail plate. Carbon monoxide toxicity may present with a red lunula.

Question 11

11. A patient with yellow nail syndrome should be evaluated for systemic disease involving which organ system?

A. Hepatic
B. Renal
C. Pulmonary
D. Cardiovascular
E. Hematologic

Answer 11

Correct choice: C. Pulmonary

Explanation: Yellow nail syndrome is characterized by slowly growing, yellow nails that are excessively curved. The lunulae and cuticles are absent. It has been associated with a array of pulmonary diseases including tuberculosis, asthma, pleural effusion, bronchiectasis, sinusitis, bronchitis, and COPD. Yellow nail syndrome is not associated with abnormalities in the other listed organ systems.

Question 12

12. A patient is diagnosed with Netherton’s syndrome and has invagination of the distal hair shaft into the cup formed by the proximal hair shaft also known as:

A. Trichorrhexis invaginata
B. Trichorrhexis nodosum
C. Trichoschisis
D. Trichothiodystrophy
E. Pili torti

Answer 12

Correct choice: A. Trichorrhexis invaginata

Explanation: Patients with Netherton’s syndrome have trichorrhexis invaginata. This syndrome also includes ichthyosis linearis circumflexa, atopy, trichorrhexis nodosa. They have a mutation in the SPINK5 gene with mutation in the serine protease inhibitor.

Question 13

13. The hair finding characterized by an invagination of the distal hair shaft into the cup formed by the proximal hair shaft is:

A. Trichoschisis
B. Pili torti
C. Pili triangulati
D. Trichorrhexis nodosa
E. Trichorrhexis invaginata

Answer 13

Correct choice: E. Trichorrhexis invaginata

Explanation: The above description is that of trichorrhexis invaginata, which is characteristic of Netherton syndrome. Other findings include: ichthyosis linearis circumflexa and atopy. It is caused by the mutation of the serine protease inhibitor, SPINK5. Trichoschisis hairs are broken transversely, pili torti hairs have a twisted appearance, pili triangulati have a triangular appearance on cross section, and trichorrhexis nodosa demonstrates a broken broomstick appearance.

Question 14

14. This hair disorder and mutations in ATP7A (MNK) gene are found in what genodermatosis?

A. Bjornstad syndrome
B. Menke’s kinky hair syndrome
C. Crandall’s syndrome
D. Bazex’s follicular atrophoderma
E. Trichothiodystrophy

Answer 14

Correct choice: B. Menke’s kinky hair syndrome

Explanation: Menke’s kinky hair syndrome is characterized by pili torti and mutations in APT7A (MNK) gene. Bjornstad syndrome, Crandall’s syndrome and Bazex’s follicular atrophoderma are associated with pili torti but are not associated with this mutation. Follicular atrophoderma can be associated with Bazex–Dupré–Christol and Conradi–Hünermann–Happle syndromes.
In Bjornstad sydrome, the hearing loss usually becomes evident very early in life, often in the first year. Pili torti, a condition in which the hair shaft is flattened and twisted, makes the hair very brittle and patients develop hair loss in the first two years of life. Björnstadsyndrome is caused by mutations in the BCS1L gene. Crandall syndrome is a very rare congenital disorder characterized by progressive sensorineural hearing loss, hair loss associated with pili torti, and hypogonadism demonstrated through low levels of luteinising hormone and growth hormone.
Trichothiodystrophy (TTD) is an autosomal recessive inherited disorder characterised by brittle hair and intellectual impairment.

Question 15

15. A six year old girl presents after being sent home from school for “lice”. On examination, there are structures on the hair which are freely slide along the hair shaft. What is the most likely diagnosis?

A. Pediculosis
B. Hair casts
C. Trichoptilosis
D. Monilethrix
E. Pili annulati
c

Answer 15

Correct choice: B. Hair casts

Explanation: Hair casts present most commonly in young girls between the age of 2 and 8 years old. These casts are freely sliding along the hair shaft and are not adherent. Hair casts must be distinguished from the nits of pediculosis capitis, which are adherent to the hair and do not slide. Trichoptilosis are “split ends” that form from chemical or physical damage to the hair. Monilethrix describes a beaded appearance of ahir due to periodic thinning of the hair shaft. Pili annulati refers to alternating light and dark bands seen in the hair shaft with reflected light.

Question 16

16. What is the gene defect that causes red hair?

A. Tyrosinase
B. P protein
C. Melanocortin 1 receptor
D. Tyrosine aminotransferase
E. Tyrosinase-related protein 1
c

Answer 16

Correct choice: C. Melanocortin 1 receptor

Explanation: Defects in melanocortin 1 receptor lead to the phenotypic appearance of red hair. Individuals with red hair have increased ratio of pheomelanin to eumelanin and are at increased risk for melanoma. The other genes are not associated with red hair.

Question 17

17. A 70-year-old female develops green-black to green-blue discoloration of her great toenail. She works as a janitor at a seaquarium and thinks she may have developed the discoloration due to frequent and prolonged exposure to water. Which of the following is not a treatment option for green nail syndrome (chloronychia)?

A. Ciprofloxacin 500 mg PO BID x 2 weeks
B. Topical application of 2% sodium hypochlorite
C. Avoidance of bleach
D. Tobramycin solution for 1-4 months
E. Removal of the affected nail

Answer 17

Correct choice: C. Avoidance of bleach

Explanation: Avoidance of bleach is not recommended as a treatment option for green nail syndrome. In green nail syndrome (chloronychia), the nail develops green–black to green–blue discoloration due to pyocyanin, a blue–green pigment produced by P. aeruginosa. Predisposing factors include frequent or prolonged exposure to water, excessive use of detergents and soaps, nail trauma and other causes of onycholysis. The diagnosis of green nail syndrome is usually clinical; if necessary, it can be confirmed by Gram stain and culture of exudate and nail fragments. The differential diagnosis includes a subungual hematoma, melanocytic nevus, melanoma, and Aspergillus infection. Treatment
involves avoidance of predisposing factors, clipping the nail, and topical application of 2% sodium hypochlorite (household bleach diluted 1:4), a quinolone, or aminoglycoside (e.g. tobramycin) solution for 1–4 months. In refractory cases, removal of the affected nail may be required.
1 - Ciprofloxacin 500 mg PO BID x 2 weeks: Treatment of green nail syndrome involves avoidance of predisposing factors, clipping the nail, and topical application of 2% sodium hypochlorite (household bleach diluted 1:4), a quinolone, or aminoglycoside (e.g. tobramycin) solution for 1–4 months.
2 - Topical application of 2% sodium hypochlorite: Treatment of green nail syndrome involves avoidance of predisposing factors, clipping the nail, and topical application of 2% sodium hypochlorite (household bleach diluted 1:4), a quinolone, or aminoglycoside (e.g. tobramycin) solution for 1–4 months.
4 - Tobramycin solution for 1-4 months: Treatment of green nail syndrome involves avoidance of predisposing factors, clipping the nail, and topical application of 2% sodium hypochlorite (household bleach diluted 1:4), a quinolone, or aminoglycoside (e.g. tobramycin) solution for 1–4 months.
5 - Removal of the affected nail: In refractory cases of green nail syndrome, removal of the affected nail may be required.

Question 18

18. This medication is a source of anagen effluvium and can cause abrupt shedding of hair:

A. Colchicine
B. Hydrochlorothiazide
C. Beta blockers
D. Calcium channel blockers
E. Procainamide

Answer 18

Correct choice: A. Colchicine
Explanation: Colchicine, mercury intoxication, thallium poisoning and severe protein deficiency can cause anagen effluvium. Hairs are usually broken and not shed.
c

Question 19

19. A 64-year-old man admitted for an exacerbation of his severe congestive heart failure exhibits blanchable white horizontal lines beneath the nail plates of all digits. What is the most likely disorder:

A. Beau’s lines
B. Mee’s lines
C. Muehrcke’s lines
D. Onychophagia
E. Dolichonychia

Answer 19

Correct choice: C. Muehrcke’s lines

Explanation: Muehrcke’s lines disappear with squeezing of the nail and are due to disorders of low albumin. Beau’s lines are transverse indented nail plate furrows caused by temporary growth arrest of the nail matrix, often due to chemotherapy or other stressful events and illnesses. Mee’s lines are transverse white lines affecting all nails and growing out with the nail; there is no associated indentation. Mee’s lines may be caused by arsenic poisoning, rheumatic fever, or other systemic diseases. Oncyhophagia refers to nail biting. Dolichonychia exhibits long, slender nails, and is associated with Ehlers-Danlos and Marfans.

Question 20

20. Patients presenting with horizontal parallel ridges on the nail, often accompanied by an absent cuticle have:

A. Habit tic deformity
B. Elkonyxis
C. Dolichonychia
D. Heller’s median canaliform dystrophy
E. Hapalonychia

Answer 20

Correct choice: A. Habit tic deformity

Explanation: Patients with horizontal parallel ridges, often accompanied by an absent cuticle, is called habit tic deformity. It is caused by trauma to the cuticle. Patients can have anxiety and obsessive compulsive disorder associated with this finding. Elkonyxis are large 2-3 mm pits on the nail. Dolichonychia are long nails associated with Marfan’s or Ehlers-Danlos syndrome.
Heller’s median canaliform dystrophy are longitudinal fissures with oblique lines caused by trauma to the proximal nail (not the cuticle as in habit tic deformity). Hapalonychia are soft nails caused by a nail matrix defect

Question 21

21. What condition most likely describes a hair defect with ruffled cuticle in 4-year old female with blonde hair?

A. Pili torti
B. Pili annulati
C. Wooly hair
D. Loose anagen hair syndrome
E. Monilethrix

Answer 21

Correct choice: D. Loose anagen hair syndrome

Explanation: Loose anagen hair syndrom occurs primarily in young, females with blond hair. The defect results in early keratinization of the inner root sheath which leads to poor anchoring. Examination of hair shaft reveals ruffled proximal cuticle. The other choices do not occur in young females with blonde hair.

Question 22

22. How long do hairs stay in telogen before they are released?

A. 3-4 weeks
B. 3-4 months
C. 3-4 years
D. 3-4 days
E. 3-4 hours

Answer 22

Correct choice: B. 3-4 months

Explanation: Hairs remain in telogen phase for 3-4 months before they are released.
The length of anagen phase varies for hairs of different parts of the body and determines the ultimate length of the hair. Scalp hair may remain in anagen for 3-5 years. The duration of catagen phase is typically 3-4 weeks.

Question 23

23. Blue lunulae are characteristic of which disease?

A. Yellow nail syndrome
B. Wilson’s disease
C. Rubenstein-Taybi syndrome
D. Hypertrophic pulmonary osteoarthropathy
E. Clubbing

Answer 23

Correct choice: B. Wilson’s disease

Explanation: Wilson’s disease is associated with characteristic blue lunulae. The other listed conditions do not typically exhibit blue lunulae.

Question 24

24. A 52 year old male with diabetes mellitus presents with a greater than 3 month history of recurrent warmth, erythema, and slight pain surrounding the first toenail of the right foot. What is the most likely diagnosis?

A. Acute Paronychia

B. Orf
C. Contact Dermatitis
D. Chronic Paronychia
E. Herpetic Whitlow

Answer 24

Correct choice: D. Chronic Paronychia

Explanation: Paronychia is an inflammatory reaction involving the folds of skin surrounding the nail. It can be characterized as acute or chronic. Acute paronychia is usually due to direct or indirect trauma followed by separation of the eponychium from the nail plate, the causative bacteria usually Staph aureus and Strep pyogenes. The treatment includes culturing the skin, systemic antibiotics, and draining of abscess if necessary. Chronic paranochya is most likely a contact reaction to irritants or allergens. It is characterized by inflammation of the proximal nail fold with erythema, edema and absence of the cuticle. The most frequently recovered organism is Candida. It was originally thought to be result of Candida infection and treatment was based upon antifungal therapy with minimal-moderate response. Newer studies suggest that it is actually dermatitis of the nail fold colonized by Candida. Treatment now is based on topical anti-inflammatory steroids.
Contact dermatitis, orf, and herpetic whitlow are not examples of paronychia disease.

Question 25

25. Twenty-nail dystrophy, nail plate splitting and pterygium formation are nail changes seen in: A. Lichen Planus B. Darier's disease C. Psoriasis D. Scleroderma E. Dermatomyositis

Answer 25

Correct choice: A. Lichen Planus Explanation: Twenty-nail dystrophy, nail plate splitting and pterygium formation are nail changes associated with Lichen Planus. Darier's disease is associated with longitudinal red and white streaks of the nail plate and V-shaped knicking distally. Psoriasis is associated with many nail abnormalities including pitting, onycholysis and oil-spots. Scleroderma may cause nail fold capillary dilation and destruction while patients with dermatomyositis may exhibit nail fold telangiectasias and frayed cuticles.

Question 26

26. Pseudo-pili annulati with light reflection off periodic flattening of the hair shaft is seen in: A. Blond hair B. Red hair C. Brown hair D. Black hair E. White hair

Answer 26

Correct choice: A. Blond hair Explanation: Pseudo pili annulati is seen in blond hair, due to the light reflection off the periodic flattening of the hair shaft. Pili annulati is the alternating of light and dark bands, secondary to air spaces, seen in normal light as opposed to "tiger-tail" hair in trichothiodystrophy, seen only under polarized light. Pseudo pili annulati is not seen in red, brown, black, or white hair. c

Question 27

27. Björnstad syndrome is the combination of pili torti and which of the following features? A. Lentigines B. White forelock C. Hypogonadism D. Deafness E. Seizures

Answer 27

Correct choice: D. Deafness Explanation: Deafness and pili torti are the two cardinal features of Björnstad syndrome.

Question 28

28. Yellow nail syndrome has been associated with use of which of the following treatments for Rheumatoid Arthritis? A. D-penicillamine B. Methotrexate C. Infliximab D. Etanercept E. Adalimumab

Answer 28

Correct choice: A. D-penicillamine Explanation: D-penicillamine has been associated with the development of yellow nail syndrome in patiens with rheumatoid arthritis.The other options are used to treat RA, but have not been associated with development of this syndrome

Question 29

29. Muehrcke's nails are most often associated with: A. Hyperthyroidism B. Hypothyroidism C. Hypoalbuminemia D. Hyperalbuminemia E. Chronic renal failure

Answer 29

Correct choice: C. Hypoalbuminemia Explanation: Muehrcke's nails are most associated with hypoalbuminemia. They represent transverse double white lines that are the abnormality of the vascular bed, probably a localized edematous state secondary to the hypoalbuminemia. The remainder of the answer choices are incorrect.

Question 30

30. The visible portion of the nail matrix is called the: A. Eponychium B. Lunula C. Cuticle D. Hyponychium E. Nail plate

Answer 30

Correct choice: B. Lunula Explanation: The nail unit is comprised of six major components including the nail matrix, nail plate, cuticle, nail bed, anchoring portion, and the framing portion (proximal/lateral/distal folds). The lunula is a component of the distal matrix. It is grayish white because of the nature of the keratinization of its epithelium. The other answer choices do not include the visible portion of the nail matrix.

Question 31

31. A patient with synophrys, dystopia canthorum, and heterochromia iridum irides likely has which of the following hair abnormalities? A. Diffuse thinning B. Scarring alopecia C. Trichorrhexis invaginata D. Trichorrhexis nodosa E. White forelock

Answer 31

Correct choice: E. White forelock Explanation: White forelock is a common feature of Waardenburg syndrome. Waardenburg syndrome is a rare genetic disorder characterized by deafness and abnormal pigmentation, such as: * White forelock * Hypopigmented (pale) patches of skin * Heterochromia iridis (differently colored eyes) Waardenburg syndrome may also be associated with musculoskeletal defects and Hirschsprung syndrome.

Question 32

32. Acute paronychia is most commonly caused by which of the following organisms? A. Candida albicans B. Candida tropicalis C. Pseudomonas aeruginosa D. Staphylococcus aureus E. Trichophyton rubrum

Answer 32

Correct choice: D. Staphylococcus aureus Explanation: Staphylococcus aureus is the most common cause of acute paronychia. Candida albicans commonly causes chronic paryonychia. Pseudomonas causes green nail. Trichophyton rubrum may cause onychomycosis.

Question 33

33. Eyebrow growth: A. Is androgen-dependent in men but not in women B. Is regulated by dehydroepiandrosterone but not androstenedione C. Is not androgen-dependent D. Requires conversion of testosterone to dihydrotestosterone in the hair follicle E. Is regulated only by testosterone and dihydrotestosterone

Answer 33

Correct choice: C. Is not androgen-dependent Explanation: Eyebrows, eyelashes, and vellus hair are not androgen-dependent, thus there is no difference between these areas of hair growth in men and women. The other answer choices listed are incorrect.

Question 34

34. What is the most likely diagnosis? A. Bjornstad syndrome B. Trichothiodystrophy C. Uncombable hair syndrome D. Monilethrix E. Netherton's syndrome

Answer 34

Correct choice: D. Monilethrix Explanation: This is an image of monilethrix, which upon trichoscopy reveals a beaded appearance of hair due to periodic thinning of the hair shaft.The other answer choices do not feature monilethrix as a clinical finding

Question 35

35. A 7-year old girl presents to clinic with onychomadesis afflicting all nails. Her mother reports that she has been healthy other than a self-limited illness two months prior. What is the most likely causative etiology: A. Rheumatic fever B. Kawasaki disease C. Systemic T cell lymphoma D. Yellow nail syndrome E. Hand, foot, mouth disease

Answer 35

Correct choice: E. Hand, foot, mouth disease Explanation: Coxsackievirus, the virus most associated with hand, foot, mouth disease, is a well- known cause of onychomadesis in children.Rheumatic fever has been associated with Mee’s lines of the nail. Yellow nail syndrome is associated with yellow nail plates, edema, and lung disease. The other answer choices are not commonly associated with nail disease.

Question 36

36. This term describes transverse white lines that affect all the nails and can be from arsenic poisoning, leprosy, and systemic disease. This nail defect is called: A. Mee's lines B. Plummer's nails C. Muehrcke's lines D. Terry's nails E. Lindsay's nails

Answer 36

Correct choice: A. Mee's lines Explanation: Transverse white lines that affect ALL the nails and grow out with nail growth are called Mee's lines. They are caused by parakeratosis of the ventral nail plate. They are associated with arsenic poisoning, rheumatic fever, congenital heart failure, leprosy, and systemic disease. Plummer's nails are onycholysis from hypothyroidism. Muehrcke's lines are double transverse white lines occuring in the setting of nephrotic syndrome, low albumin, liver disease, and malnutrition. Since they occur from an abnormal vascular bed, the lines disappear with squeezing of the nail. Terry's nails are leukonychia of all but the distal 2 mm of the nail. It occurs in the setting of cirrhosis, congenital heart disease, and diabetes. Lindsay's nails (half and half nails) presents as white proximal nail and normal distal nail. It occurs from nailbed edema in the setting of chronic renal failure.

Question 37

37. This is the scalp of a 7 year-old-girl. What is the most likely diagnosis? A. Alopecia areata B. Triangular alopecia C. Frontal fibrosing alopecia D. Central centrifugal cicatricial alopecia E. Morphea

Answer 37

Correct choice: E. Morphea Explanation: The image depicts a linear, erythematous, shiny, atrophic patch of scarring alopecia (note the loss of follicular ostia). This is most consistent with linear morphea (en coup de sabre), which can present in children and adults. Alopecia areata and triangular alopecia are non-scarring forms of alopecia. Frontal fibrosing alopecia and central centrifugal cicatricial alopecia are types of scarring alopecia, but they do not present with linear, erythematous, shiny, atrophic patches.

Question 38

38. Which is the most effective for the treatment of onychomycosis? A. Oral terbinafine B. Terbinafine cream C. Econazole cream D. Clobetasol ointment E. Oral ketoconazole

Answer 38

Correct choice: A. Oral terbinafine Explanation: Terbinafine, a squalene epoxidase inhibitor, is the treatment of choice for onychomycosis. The treatment is with 250 mg by mouth daily for 12 weeks for toenails and 6 weeks for fingernails. Other treatment options include oral itraconazole. Topicals such as terbinafine cream and econazole cream are good choices for tinea pedis, but are not successful in treating onychomycosis. Topical corticosteroids such as clobetasol ointment are not effective in treating fungal infections. Oral ketoconazole is ineffective for onychomycosis.

Question 39

39. Mee's lines are: A. Double white transverse lines from abnormal vascular bed B. Brownish macules beneath the nail plate C. Local or diffuse hyperkeratotic tissue that develops on the lateral or proximal nail folds D. Transverse white lines that affect all nails, grow out with nail growth E. Vertical black lines on single or multiple nails

Answer 39

Correct choice: D. Transverse white lines that affect all nails, grow out with nail growth Explanation: Mee's lines are transverse white lines that affect all nails, grow out with nail growth. They are seen in arsenic poisoning, rheumatic fever, congenital heart failure, leprosy and with significant systemic disease. Brownish macules beneath the nail plate are oil spots, seen in psoriasis. Onychophosis is the local or diffuse hyperkeratotic tissue that develops on the lateral or proximal nail folds. The double white transverse lines from abnormal vascular bed are Muehrcke's lines which are caused by a nephrotic syndrome, low albumin, liver disease or malnutrition.

Question 40

40. This finding may be seen in all of the following EXCEPT: A. Menkes disease B. Trichothiodystrophy C. Crandall syndrome D. Arginosuccinic aciduria E. Netherton's syndrome

Answer 40

Correct choice: C. Crandall syndrome Explanation: This image depicts trichorrhexis nodosa (TN), which describes hair with an incomplete fracture with frayed ends resembling two pain brushes against each other. Pili torti (twisting and flattening of the hair) is seen in Crandall syndrome, not TN. TN is seen in the remaining answer choices.

Question 41

41. Black nail is caused by: A. Trichophyton mentagrophytes B. Proteus mirabilis C. Pseudomonas spp. D. Staphylococcus aureus E. Hortaea werneckii

Answer 41

Correct choice: B. Proteus mirabilis Explanation: Nail infections with Proteus mirabilis. T. mentag causes white discoloration of the nail and Pseudomonas will cause a green or yellow/ green discoloration. Hortaea werneckii causes tinea nigra. S. aureus does not cause black nails.

Question 42

42. Mutations in hair keratins hHB6 and hHB1 cause this characteristic finding pictured below – name the condition: A. Trichoptilosis B. Hair casts C. Tiger-tail hair D. Trichoschisis E. Monilethrix

Answer 42

Correct choice: E. Monilethrix Explanation: Mutations in the hair keratins, hHB6 and hHB 1 cause monilethrix. Hair casts (HCs) are thin, elongated, firm, whitish cylindrical concretions which ensheath the hair shaft and can be easily dislodge. They are of two different types: peripilar and parakeratotic keratin cast. Due to its clinical resemblance to pediculosis capitis, the entity is also known as pseudonits. Trichoschisis is fractured hair. Trichoptilosis, schizotrichia, and informally split ends, is the splitting or fraying of the hair-shaft due to excessive heat and mechanical stress. Polarizing light microscopy is the gold standard for detecting the characteristic light and dark bands, the so-called “tiger-tail” or “zigzag” pattern, which has been incorrectly referred to as alternating birefringence.

Question 43

43. A patient presents with 20 nails with absent cuticles and lunulae, slow growth, dystrophic shape and a yellow hue. Which of the following findings is/are associated? A. Upper extremity edema B. Cirrhosis C. Pleural effusions D. Chest pain E. Pulmonary fibrosis

Answer 43

Correct choice: C. Pleural effusions Explanation: Yellow Nail syndrome has been associated with lower extremity edema, bronchiectasis and pleural effusions. The other listed findings are not part of Yellow Nail syndrome.

Question 44

44. Chronic paronychia is most commonly caused by which of the following organisms? A. Candida albicans B. Candida tropicalis C. Pseudomonas aeruginosa D. Staphylococcus aureus E. Trichophyton rubrum

Answer 44

Correct choice: A. Candida albicans Explanation: Candida albicans is the most common pathogen associated with chronic paronychia. Staph aureus is the most common pathogen associted with acute paronychia. Pseudomonas causes green nail. Trichophytan spp cause onychomycosis.

Question 45

45. A 36-year-old woman presents with hair loss. Examination of a single follicle reveals multiple hair shafts protruding, each surrounded by its own cuticle. What is the most likely diagnosis: A. Pili multigemini B. Pili bifurcati C. Trichoptilosis D. Trichoschisis E. Trichothiodystrophy

Answer 45

Correct choice: A. Pili multigemini Explanation: Pili multigemini refers to a rare disorder of multiple divided hair matrices, with multiple hair shafts each with their own cuticle all arising from one follicle. Pili bifucarti exhibits hair shaft bifurcation into short segments along hair shafts, with each segment covered with it’s own cuticle. Trichoptilosis refers to “split ends” of the distal portion of the hair shaft. Trichoschisis is a clean fracture of the hair shaft. Trichothiodystrophy is a disorder of beaded hair when viewed with polarizing light.

Question 46

46. Which of the following is the predominant dermatoscopic finding seen in alopecia areata? A. Diffuse white knots and a brush-pattern B. Perifollicular arborizing vessels C. Yellow dots D. Reduction of follicular ostia E. Small oval nodes

Answer 46

Correct choice: C. Yellow dots Explanation: Dermatoscopic findings of alopecia areata include yellow dots, dystrophic hair shafts features, and hypopigmented vellus hairs. Diffuse white knots and a brush pattern is seen in trichorrhexis nodosa. In lichen planopilaris, a reduction to absence of follicular ostia, perifollicular scale and arborizing vessels, pigmented networks, and white to blue-gray dots can be observed. Small oval nodes are seen in monilethrix

Question 47

47. Fraying of the cuticles occurs in which condition? A. Dermatomyositis B. Systemic sclerosis C. Lupus erythematosus D. Hypothyroidism E. Lead poisoning

Answer 47

Correct choice: A. Dermatomyositis Explanation: Dermatomyositis can be associated with fraying of the cuticles (Samitz sign). The other listed conditions do not lead to changes of the cuticle.

Question 48

48. A patient with end stage renal disease and on dialysis has an opaque white appearance of half of the nail. This is called: A. Lindsay's nails B. Terry's nails C. Birt Hogg nails D. Riley's nails E. Hutchinson's nails c

Answer 48

Correct choice: A. Lindsay's nails Explanation: This finding associated with end stage renal disease where half of the nail is opaque is called Lindsay's nail. Patients with cirrhosis or hypoalbuminemia that have whitening of the proximal two third of the nail with a 1/3 that is red is called Terry's nails.

Question 49

49. Which genodermatosis characterized by broad thumbs and this nail disorder pictured below? A. Rothmund-Thompson B. Tuberous sclerosis C. Pachyonychia congenita D. Proteus syndrome E. Rubenstein-Taybi syndrome

Answer 49

Correct choice: E. Rubenstein-Taybi syndrome Explanation: Rubensteom-Taybi syndrome is characterized by broad thumbs and broad nails or brachyonychia. This syndrome is characterized by mutations in CREB-binding protein. Rubinstein-Taybi syndrome is characterized by broad lateral faces, shoulders and back, thick eyebrows and trichomegaly. Patients also have a tendency for mid facial vascular stains, keloids, pilomatricomas, broad thumbs/halluces, beaked noses, high-arched palates, short statures.

Question 50

50. Pili trianguli et canaliculi is the characteristic hair finding in which of the following disorders? A. Monilethrix B. Pili annulati C. Naxos syndrome D. Anhidrotic ectodermal dysplasia E. Uncombable hair syndrome

Answer 50

Correct choice: E. Uncombable hair syndrome Explanation: Pili trianguli et canaliculi, characterized by a triangular hair shaft with a central longitudinal groove, is the finding seen in uncombable hair syndrome. The other listed answer choices are incorrect.

Question 51

51. A 24-year-old man with Lesch-Nyhan syndrome is most likely to present with what nail disorder: A. Beau’s lines B. Mee’s lines C. Muehrcke’s lines D. Onychophagia E. Dolichonychia

Answer 51

Correct choice: D. Onychophagia Explanation: Oncyhophagia refers to nail biting. Patient’s with Lesch-Nyhan syndrome, caused by defective HGPRT enzyme, are prone to self-mutilating behaviors, including onychophagia. Beau’s lines are transverse indented nail plate furrows caused by temporary growth arrest of the nail matrix, often due to chemotherapy or other stressful events and illnesses. Mee’s lines are transverse white lines affecting all nails and growing out with the nail; there is no associated indentation. Mee’s lines may be caused by arsenic poisoning, rheumatic fever, or other systemic diseases. Muehrcke’s lines disappear with squeezing of the nail and are due to disorders of low albumin. Dolichonychia exhibits long, slender nails, and is associated with Ehlers-Danlos and Marfans.

Question 52

52. Menkes kinky hair syndrome is associated most commonly with: A. Trichorrhexsis invaginata B. Trichostasis spinulosa C. Pili multigemini D. Pili torti E. Plica neuropathica

Answer 52

Correct choice: D. Pili torti Explanation: The X-linked recessive Menkes kinky hair syndrome is associated with multiple hair shaft abnormalities, most characteristically, pili torti. It is caused by a mutation of the transport protein mediating copper uptake from the intestine, encoded by the ATP7A gene.Trichorrhexsis invaginata is most commonly associated with Netherton syndrome. The other answer choices are not commonly associated with Menkes.

Question 53

53. This condition describes a mutation in the Hairless gene and is characterized by universal alopecia developing shortly after birth. Patients with this condition have normal eyelashes and cornified cysts over the face and neck. This describes: A. Papular atrichia B. Acquired progressive kinking C. Trichotillomania D. Woolly hair nevus E. Ichthyosis follicularis, atrichia, photophobia (IFAP)

Answer 53

Correct choice: A. Papular atrichia Explanation: Patients with papular atrichia have an autosomal recessive mutation in the hairless gene, which irreversibly destroys hair follicles. The natal hair falls out after birth, typically around 3 months, and never regrows. Patients present with normal eyelashes and cornified cysts over the face and neck. Acquired progressive kinking typically presents in a post-pubescent male with androgenetic alopecia and gradual curling and darkening of the frontal, temporal, and auricular hair regions. Trichotillomania is compulsive hair pulling which presents as irregular broken hairs within a geometric localized area. Woolly hair nevus is an isolated congenital patch of hairs with axial twisting and elliptical cross-sections. Ichthyosis follicularis, atrichia, and photophobia (IFAP) presents with absent eyebrows and eyelashes (in contract with papular atrichia). Patients also present with photophobia, punctate corneal scarring, keratoconjunctival inflammation, and nystagmus.

Question 54

54. All of the following are associated with trichorrhexis nodosa except: A. Trichothiodystrophy B. Absent argininosuccinase C. Netherton's syndrome D. Menke's kinky hair syndrome E. Bjornstad syndrome

Answer 54

Correct choice: E. Bjornstad syndrome Explanation: Bjornstad syndrome is associated with pili torti and deafness. Patients that have trichorrhexis nodosa have frayed ends resembing "broom-stick" or "paint-brush" hair. The following are associated with this condition: argininosuccinic aciduria, Menke's kinky hair syndrome, Trichothiodystrophy, and Netherton's syndrome.

Question 55

55. This term is used to describe nails with absent cuticles/lunulae and slow growth with transverse ridging: A. Yellow nail syndrome B. Unguis incartus C. Transverse white bands D. Trachyonychia E. Terry's nails

Answer 55

Correct choice: A. Yellow nail syndrome Explanation: Yellow nail syndrome presents with absent cuticles/lunulae, slow growth, dystrophic shape, and transverse ridging. This syndrome is associated with lower extremity with lymphedema and bronchiectasis and pleural effusions which can occur in the setting of TB, asthma, and cancers. It is also associated with D-penicillamine and bucillamine use in patients with rheumatoid arthritis. Unguis incartus is also known as ingrown nails. Transverse white bands typically affect 1-2 nails and are caused by trauma of the nail matrix. Trachyonychia presents as rough nails. Terry's nails present from a defect in the nail bed caused by cirrhosis, congenital heart failure, and diabetes. These present as leukonychia of all but the distal 2 mm of the nail.

Question 56

56. Koilonychia is most commonly seen in association with: A. Renal disease B. Progressive systemic sclerosis C. Alopecia areata D. Impaired iron metabolism E. Pulmonary disease

Answer 56

Correct choice: D. Impaired iron metabolism Explanation: In koilonychia (spoon nails), the nail is a concave shape with raised lateral edges. Iron-deficiency anemia and Plummer-Vinson syndrome have been associated with koilonychia. Involvement of only the first three fingernails is suggestive of an occupational cause. In early infancy, koilonychia is a normal finding. Koilonychia is also seen in hemochromatosis, an autosomal recessive condition associated with increased intestinal iron absorption and increased iron deposition leading to metallic-grey hyperpigmentation of the skin, hepatomegaly, diabetes, hypogonadism, polyarthritis, and cardiac abnormalities. Koilonychia is not associated with the other listed conditions.

Question 57

57. A 6-year old girl with atopic dermatitis presents with patchy non-scarring alopecia and nail pitting. You make the diagnosis of alopecia areata. What portion of the nail unit is responsible for her nail plate pitting: A. Hyponychium B. Proximal nail plate C. Proximal nail matrix D. Distal nail matrix E. Eponychium

Answer 57

Correct choice: C. Proximal nail matrix Explanation: The proximal nail matrix produces the dorsal nail plate. Defective keratinization in this region is therefore responsible for the pitting seen in alopecia areata, psoriasis, eczema, and other cutaneous diseases. The distal nail matrix produces the ventral nail plate and is visible as the lunula.

Question 58

58. The term used to describe hair shafts in anagen effluvium that are abruptly thinned, forming constrictions and breaking off at the skin surface is: A. Pohl-Pinkus B. Trichoptilosis C. Monilethrix D. Trichoschisis E. Trichorrhexis Nodosa

Answer 58

Correct choice: A. Pohl-Pinkus Explanation: Pohl-Pinkus constriction is used to describe abruptly thinned hair shafts. This is seen in anagen effluvium, secondary to a toxic insult. The shedding of anagen hairs are always abnormal. This is analogous to Beau's lines in nails. Trichoptilosis or "split ends" occur secondary to chemical or physical damage. Monilethrix has elliptical nodes at 0.7-1 mm intervals and hair fractures at the nodes. Trichoschisis are clean transverse fractures of the hair follicle. Trichorrhexis Nodosa are frayed ends resembling broom-sticks or paint brushes.

Question 59

59. A 42 year-old woman presents with the complaint of excess hair growth on her face. She has normal menses and has recently had her "annual" exam and the note relates normal sized ovaries. What is the most logical next step? A. Check plasma levels of androstenedione and testosterone B. Send a 21-hydroxylase enzyme deficiency test C. Biopsy from the most affected area D. Refer her to endocrinology E. Order a CT of the abdomen

Answer 59

Correct choice: A. Check plasma levels of androstenedione and testosterone Explanation: Women with idiopathic hirsutism will have evidence of androgen excess but with normal menses, normal-sized ovaries and no evidence of tumors of adrenal or ovary and normal adrenal function. They will often have slight elevations of plasma androstenedione and testosterone. Check the blood levels of the plasma steroids would be a logical first step. The remained of the answer choices should be reserved until a basic hormonal workup is first performed for evaluation to guide further management.

Question 60

60. Signs of virilization include all of the following EXCEPT: A. Centripetal obesity B. Temporal balding C. Clitoromegaly D. Increased muscle mass in the limb girdles E. Acne

Answer 60

Correct choice: A. Centripetal obesity Explanation: Centripetal obesity is a sign of cortisol excess not of virilization. In addition to the those listed above, irregular or absent menses and deepening of the voice are signs of virilization. Temporal balding, clitoromegaly, increased muscle mass in limb girdles, and acne are all signs of virilization

Question 61

61. A patient presents with thin anterior hair and redundant, thickened skin around the neck, palms, and soles. The patient has congenital myopathy, coarse facies, facial and perianal papillomas, and cardiac and developmental disability. The mutation is in: A. HRAS gene B. KRAS gene C. BRAF gene D. MEK1 gene E. MEK2 gene

Answer 61

Correct choice: A. HRAS gene Explanation: Patients with Costello syndrome (also known as faciocutaneoskeletal syndrome) have a mutation in the HRAS gene, which is part of the Ras-MAPK pathway. It has phenotypic overlap with Cardio-facio-cutaneous Syndrome which occurs from a KRAS mutation. Costello syndrome does not occur from a KRAS, BRAF, MEK1, or MEK2 mutation.

Question 62

62. Endonyx onychomycosis is most commonly due to: A. Trichophyton rubrum B. Trichophyton tonsurans C. Trichophyton soudanense D. Scytalidium dimidiatum E. Scopuliaropsis

Answer 62

Correct choice: C. Trichophyton soudanense Explanation: Endonyx onychomycosis is most commonly due to T. soudanense, although it may also be observed with other Trichophyton species (e.g. rubrum, tonsurans). This is the equivalent of endothrix infection of the hair associated with tinea capitis.T. rubrum and T. tonsurans are less common causes of endonyx onychomycosis. Scytalidium dimidiatum and Scopuliaropsis do not typically produce endonyx onychomycosis.

Question 63

63. Lindsay's nails (distal nail normal, proximal nail white) is characteristic of: A. Chronic renal failure B. Plummer-Vinson syndrome C. Hemochromatosis D. Ectodermal dysplasia E. LEOPARD syndrome

Answer 63

Correct choice: A. Chronic renal failure Explanation: Lindsay's nails are characteristic of chronic renal failure and indicate nailbed edema. The remaining options are causes of koilonychia, a spooning defect of the nail.

Question 64

64. A 34-year-old man presents with a jet-black nail plate involving the right index finger. Hutchinson’s sign is negative and the patient denies antecedent trauma. What is the most likely etiology of the nail plate discoloration: A. Multiple myeloma B. Wilson disease C. Congenital oncychodysplasia of the index finger (COIF) D. Pseudomonas infection E. Proteus infection

Answer 64

Correct choice: E. Proteus infection Explanation: Proteus infection of the nail is associated with black discoloration of the nail plate. Multiple myeloma may be associated with anonychia. Wilson disease classically exhibits blue lunulae. COIF presents with absence of the nail plate. Pseudomonal infections of the subunguium may produce a green discoloration due to production of pyocyanin pigment.

Question 65

65. Patients that experience hypertrichosis lanuginosa acquisita can be due to all of the following except: A. Tetracyclines B. Thyrotoxicosis C. Corticosteroids D. Phenytoin E. Spironolactone

Answer 65

Correct choice: A. Tetracyclines Explanation: Tetracyclines are not associated with hypertrichosis lanuginosa aquisita. Hypertrichosis lanuginosa aquisita could be due to thyrotoxicosis, corticosteroids, phenytoin, and spironolactone. It is abrupt onset of downy, soft non-pigmented hair on the face and can be associated with glossistis and lung carcinoma.

Question 66

66. A significantly increased number of "club hairs" on a pull test is indicative of: A. Anagen effluvium B. Telogen effluvium C. Normal hair anatomy D. Alopecia areata E. Angrogenetic alopecia

Answer 66

Correct choice: B. Telogen effluvium Explanation: Telogen hairs are characteristically club shaped. An increased number of telogen hairs on hair pull would be suggestive of telogen effluvium. Anagen hairs have a curled appearance at the root. While a small number of telogen hairs are normal, a significantly increased number would not be normal hair anatomy. The remainder of the answer choices do not exhibit increased numbers of club hairs on pull test.

Question 67

67. The most common cause of a longitudinal groove of the nail plate is: A. Myxoid cyst B. Verruca vulgaris C. Psoriasis D. Onychomycosis E. Paronychia

Answer 67

Correct choice: A. Myxoid cyst Explanation: A myxoid cyst is not a true cyst as it is devoid of cyst lining. They usually occur in an acral location and are the most common cause of a longitudinal groove of a nail plate. The other listed answers are less common causes of a longitudinal groove of the nail plate.

Question 68

68. A patient with diffuse hair loss developing after a thallium scan likely has which of the following conditions? A. Anagen effluvium B. Catagen effluvium C. Telogen effluvium D. Loose anagen syndrome E. Uncombable hair syndrome

Answer 68

Correct choice: A. Anagen effluvium Explanation: Anagen effluvium can result from infusions of thallium. The other conditions have not reported to be caused by thallium infusions.

Question 69

69. Which of the following are findings associated with Nail-Patella Syndrome? A. Glomerulonephritis B. Alopecia C. Bladder diverticuli D. Photosensitivity E. Hip dysplasia

Answer 69

Correct choice: A. Glomerulonephritis Explanation: Nail patella syndrome is a rare, AD syndrome associated with nail hypoplasia, absent or hypoplastic patella, posterior iliac horns, glaucoma, hyperpigmentation of the pupillary margin or Lester iris, and glomerulonephritis and nephrotic syndrome. Glomerulonephritis and renal failure are features seen in nail patella syndrome. This syndrome is also known as HOOD syndrome or hereditary osteoonychodysplasia.

Question 70

70. A patient with wooly hair, diffuse palmoplantar keratoderma, and right ventricular arrhythmogenic cardiomyopathy likely has a mutation in which of the following genes? A. hHb6 B. Plakoglobin C. Plakophilin D. SPINK-5 E. SLURP-1

Answer 70

Correct choice: B. Plakoglobin Explanation: Naxos syndrome is caused by mutations in the plakoglobin gene. Its clinical features include wooly hair, diffuse palmoplantar keratoderma, and right ventricular arrhythmogenic cardiomyopathy. SPINK-5 is mutated in Netherton syndrome. SLURP-1 mutations have been described in Mal de Meleda. Plakophilin mutations have been described in right ventricular cardiomyopathy without keratoderma. Keratin hHb6 (keratin 86) is mutated in monilethrix.

Question 71

71. A patient presents with thickened, hypertrophied nail plates and subungual hyperatosis. The type of this condition that is associated with a keratin 6 and 16 mutation is: A. Type I B. Type II C. Type III D. Type IV E. Type V

Answer 71

Correct choice: A. Type I Explanation: Pachyonychia congenita is condition characterized by 20 nail dystrophy, subungual hyperkeratosis, pincer nails, and paronychia. Type I: Jadassohn-Lewandowsky is associated with keratin 6a and 16 mutation, focal PPK, follicular hyperkeratosis, and oral leukokeratoses that are not premalignant. Type II (Jackson-Lawler) syndrome involves mutations in keratins 6b and 17 and clinically resembles the type I syndrome with the additional findings of both natal teeth and steatocystoma multiplex; less severe palmoplantar keratoderma with milder or absent oral lesions Type III (Schafer-Branauer) syndrome features all the findings of the type I disease and leukokeratosis of the corneas Type IV, pachyonychia congenita tarda, applies to a late-onset of the disease during the second

Question 72

72. A 55 year old Caucasian female presents for evaluation of hair loss. Examination reveals decreased frontal scalp hair density with perifollicular erythema and scale. A punch biopsy is performed to confirm the diagnosis. Which of the following answer choices represents the most likely histologic findings from this patient? A. Vacuolar interface alteration of the epidermis with increased mucin B. Incomplete, disrupted follicular anatomy with pigment casts noted C. Band-like lymphocytic infiltrate obscuring the interface between follicular epithelium and dermis D. Peribulbar mononuclear cell infiltrate (with occasional eosinophils), with an increased number of miniaturized hairs E. A decreased total number of hairs, with loss of the sebaceous glands and residual hair shafts surrounded by mild, granulomatous inflammation

Answer 72

Correct choice: C. Band-like lymphocytic infiltrate obscuring the interface between follicular epithelium and dermis Explanation: Choice 3 represents the findings seen in frontal fibrosing alopecia (a subset oflichen planopilaris), which is the condition described. Lichen planopilaris manifests histologically with similar findings to lichen planus, with a band like lymphocytic infiltrate which is found at the dermal-epidermal junction. The infiltrate is more superficial than that of alopecia areata (choice 4). Choice 1 describes discoid lupus. Choice 2 describes trichotillomania. Choice 4 represents alopecia areata. Choice 5 represents end stage cicatricial alopecia.

Question 73

73. A patient with cirrhosis has noticed that the proximal two-thirds of his nails have become white, whereas the distal one-third of his nails have a brown-pink band. This characteristic finding is called: A. Lindsay's nails B. Mees' lines C. Beau's lines D. Terry's nails E. Muehrcke's bands

Answer 73

Correct choice: D. Terry's nails Explanation: Terry’s nails are defined as the proximal two-thirds of nails being white, whereas the distal one-third of the nails have a brown-pink band. It has been described in association with cirrhosis, hypoalbuminemia, congestive heart failure, and diabetes mellitus. Lindsay's nails (also called half-and-half nails) are associated with chronic renal disease and are defined as the proximal one-half of nails being white, whereas the distal one-half contains a red- brown zone. Mee's lines is the term used to describe the finding of transverse lines of the entire nail breadth in all nails (can be due to arsenic poisoning, trauma, medications, severe illness, or a psoriasis flare). Beau's lines describes a transverse depression in the nail plate surface, and they are associated with a severe systemic event (all nails) or trauma/disease of nail matrix (single nail). Muehrcke's bands represent transverse white bands parallel to the lunula (seen in association with hypoalbuminemia or chemotherapy).

Question 74

74. This term is used to describe the epithelium which starts at the mid distal phalanx and generates and determines the thickness of the nail plate: A. Nail matrix B. Nail plate C. Proximal nail fold D. Nail bed E. Hyponychium

Answer 74

Correct choice: A. Nail matrix Explanation: The nail matrix generates the nail plate and is responsible for the thickness of the nail plate. It keratinizes without a granular layer. The nail plate is the actual nail made up of tightly packed onychocytes. The proximal nail fold is the dorsal part of the digit that lies adjacent to nail.

Question 75

75. This hair disorder is used to describe end stage scarring alopecia with areas of hair growth that look like "footprints in the snow": A. Pseudopelade of Brocq B. Lichen planopilaris C. Aplasia cutis congenita D. Folliculitis decalvans E. Follicular mucinosis

Answer 75

Correct choice: A. Pseudopelade of Brocq Explanation: Pseudopelade of Brocq is an end stage scarring alopecia that presents as an oval or irregularly shaped patch of alopecia with areas of hair growth that look like footprints in the snow. Lichen planopilaris presents as alopecia with perifollicular erythema. Aplasia cutis congenita most commonly presents on the scalp as a focal absence of epidermis. Folliculitis decalvans presents with inflammation, boggy induration, and pustules on the scalp. Follicular mucinosis presents with alopecia or broken hairs on the scalp or beard area. It typically occurs primary or secondary to CTCL.The nail bed is the thin epithelium immediately beneath the nail plate. This has no granular layer. The hyponychium is the epithelium that lies on the volar surface of the digit.

Question 76

Which of the following is not associated with increased fragility? A. Trichorrhexis invaginata B. Monilethrix C. Pili torti D. Trichorrhexis nodosa E. Pili multigemini

Answer 76

Correct choice: E. Pili multigemini Explanation: All of the following are associated with increased fragility except for pili multigemini. It is a malformation characterized by the presence of bifurcated or multiple divided hair matrices and papillae, giving rise to the formation of multiple hair shafts within the individual follicles. Trichorrhexis invaginata presents as the intussusception of the proximal hair shaft into the distal end, which increases hair fragility. Monilethrix presents with alternating normal thickness and constrictions, which increases hair fragility. Pili torti presents with a flattened hair shaft which twists on its own axis, increasing hair fragility. Trichorrhexis nodosa presents with frayed ends resembling a broom stick or paint brush, also with increased hair fragility.

Question 77

77. A 32-year-old male presents with progressive curliness of the temporal scalp hairs that seem to precede hair loss. What is the most likely associated etiology? A. Secondary syphilis B. Systemic tuberculosis C. Lichen planopilaris D. Hodgkin’s lymphoma E. HIV

Answer 77

Correct choice: E. HIV Explanation: HIV and retinoids are associated with acquired progressive kinking of the hair. This syndrome presents with gradual curling and darkening of hair in the frontal and temporal regions of the scalp, followed by androgenetic alopecia. Secondary syphilis may present as a non-scarring alopecia, typically in a diffuse pattern.

Question 78

78. The diagnosis is A. Psoriasis B. Alopecia areata C. Onychomycosis D. Lichen planus E. Tetracycline-induced photo-onycholysis

Answer 78

Correct choice: D. Lichen planus Explanation: Lichen planus-related nail changes seen here include thinning of the nail plate with onychorrhexis, ridging and pterygium formation. While the other listed conditions can affect the nails, this clinical picture, especially pterygium formation, most likely represents LP.

Question 79

79. Hirsutism is the development of terminal body hair in women in areas where this type of hair is not normally found. All of the following drugs are associated with hirsutism except: A. Minocycline B. Phenytoin C. Minoxidil D. Diazoxide E. Cyclosporine

Answer 79

Correct choice: A. Minocycline Explanation: All of the above are associated with hirsutism except minocycline. They cause hirsutism without virilization or defeminization. Minocycline does not cause hirsutism.

Question 80

80. Yellow nails can be seen in each of the following conditions except: A. Bronchiectasis B. Hyperlipidemia C. Pleural effusion D. Thyroid disease E. Chronic sinusitis

Answer 80

Correct choice: B. Hyperlipidemia Explanation: Hyperlipidemia is not associated with nail changes. Yellow discoloration of the nail plate can be associated with bronchiectasis or other underlying pulmonary disease such as COPD or pleural effusion. Other associated conditions include lymphedema, rheumatoid arthritis, thyroid disease, malignancies, penicillamine, and chronic sinusitis or bronchitis.

Question 81

81. Which of the following syndromes does NOT have the finding shown in the image as part of the spectrum? A. Crandall B. Argininosuccinic aciduria C. Citrullinemia D. Menke's Kinky Hair E. Netherton

Answer 81

Correct choice: A. Crandall Explanation: Crandall syndrome has pili torti as its hair finding. Trichorrhexis nodosa is the fraying of hair ends so that the resemble a "broom-stick" or "paint brush". The other options all have trichorrhexis nodosa as one of the hair findings in the syndrome.

Question 82

82. A 50-year-old African American female presents with slowly progressive, symmetric hair loss centered at the crown and vertex. A scalp biopsy shows evidence of inflammation in the active, peripheral zone. Which of the following is the most common form of scarring alopecia in any population that includes a significant number of black patients? A. central centrifugal cicatricial alopecia B. telogen effluvium C. discoid lupus erythematosus D. alopecia areata E. androgenetic alopecia

Answer 82

Correct choice: A. central centrifugal cicatricial alopecia Explanation: Central centrifugal cicatricial alopecia is a slowly progressive, symmetric cicatricial alopecia centered on the crown or vertex. It is most often found in black women of African heritage. Early and mild disease can be effectively treated, even severe disease may be significantly improved with appropriate therapy. It is the most common form of scarring alopecia in any population that includes a significant number of black patients. 2 - telogen effluvium:- Telogen effluvium is characterized by increased shedding of otherwise normal telogen hair sin response to a pathologic or noral physiologic change in health status. 3 - discoid lupus erythematosus:- Lesions of discoid lupus erythematosus occur most commonly on the face, ears and scalp. Various clinical patterns of scalp involvement can be seen including scarring alopecia. 4 - alopecia areata:- Alopecia areata is a non-scarring patterned alopecia, most commonly presenting as circular areas of alopecia. It is an organ-specific autoimmune disease involving T cells. 5 - androgenetic alopecia:- Androgenetic alopecia is a genetically determine sensitivity of scalp hair follicles to adult level sof androgens. There is miniaturization of hairs in a symmetric pattern on the crown, vertex, and frontal regions.

Question 83

83. The term used to describe the thinning of the hair shaft, which forms constrictions along the hair, due to the disruption of the anagen cycle is: A. Pohl Pinkus B. Trichorrhexis invaginata C. Trichoschisis D. Monilethrix E. Pili annulati

Answer 83

Correct choice: A. Pohl Pinkus Explanation: Pohl Pinkus constriction is due to a toxic insult. This causes the disruption of the anagen cycle with thinning of the hair shaft, forming constrictions along the hair. This is analogous to Beau's lines in nails. Trichorrhexis invaginata is the invagination of the distal hair shaft into the cup formed by the proximal hair shaft, i.e. "ball and socket" or "bamboo hair", seen in Netherton's syndrome. Trichoschisis is the clean transverse fracture of the hair follicle. Monilethrix presents as elliptical nodes at 0.7-1 mm intervals in the hair. Pili annulati presents as alternating light and dark bands seen in normal light, due to air spaces in the hair shaft.

Question 84

84. The syndrome including mental and physical retardation, convulsions, episodic unconsciousness, liver enlargement, skin lesions, and dry and brittle hair showing trichorrhexis nodosa microscopically and fluorescing red. is: A. Bazex Follicular Atrophoderma B. Crandall's syndrome C. Citrullinemia D. Argininosuccinic aciduria E. Bjornstad's syndrome

Answer 84

Correct choice: D. Argininosuccinic aciduria Explanation: Argininosuccinic aciduria has the features listed as well as trichorrhexis nodosa and red fluorescence of the hair. Bazex's follicular atrophoderma and Crandall syndrome both have findings of pili torti and citrullinemia has trichorrhexis nodosa. None of the other options have red fluorescence as a feature.

Question 85

85. Argininosuccinic aciduria is characteristically associated with this hair finding –name the condition pictured below: A. Pili trianguli et canaliculi B. Trichoschisis C. Monilethrix D. Trichorrhexis nodosa E. Trichorrhexis invaginate

Answer 85

Correct choice: D. Trichorrhexis nodosa Explanation:Trichorrhexis nodosa is a finding in argininosuccinic aciduria. Trichorrhexis nodosa may be a congenital or acquired abnormality. It may be seen in children with intellectual disability and argininosuccinic aciduria or citrullinemia as well as those with tricho-hepato-enteric syndrome. Trichorrhexis invaginata, also known as bamboo hair, is commonly seen in association with ichthyosis linearis circumflexa in patients with Netherton syndrome. Monilethrix (beaded hair) is a term of Greek and Latin derivation meaning “necklace hair”. This disorder is inherited in an autosomal dominant fashion with variable expression. Monilethrix has been mapped to the epithelial keratin gene cluster on 12q11–q13, and point mutations have been found in the hair cortex-specific keratin genes KRT86 (most often) and KRT81. In addition, mutations have been found in the genes that encode KRT83 and in patients with an autosomal recessive form of monilethrix, desmoglein. Transverse fractures are known as trichoschisis. This entity is also known as pili trianguli et canaliculi and refers to a rare hair abnormality in which hair has a “spun glass” appearance due to the reflection of light from variably oriented, flattened hair surfaces. Hair is characteristically stiff and difficult to comb. Abnormal keratinization of the internal root sheath is postulated to cause the irregularly shaped hair shafts, which have a triangular shape on cross-section and a longitudinal groove that is best seen by scanning electron microscopy.

Question 86

86. A patient with psoriasis has pitting of the nails. This finding is due to involvement of which part of the nail unit? A. Proximal matrix B. Nail bed C. Hyponychium D. Proximal nail fold E. Distal matrix

Answer 86

Correct choice: A. Proximal matrix Explanation: Disease in the proximal matrix is responsible for producing pitting, onychorrhexis, and Beau’s lines. Changes in the intermediate matrix can cause leukonychia, and the distal matrix may be responsible for focal onycholysis, thinned nail plate, and erythema of the lunula.

Question 87

87. Best diagnosis of this type of cicatricial alopecia shown in attached image is: A. Chronic cutaneous lupus erythematosus B. Lichen planopilaris C. Central centrifugal cicatricial alopecia D. Pseudopelade of Brocq E. Alopecia mucinosa

Answer 87

Correct choice: C. Central centrifugal cicatricial alopecia Explanation: Central centrifugal cicatricial alopecia is a new term adopted by the NAHRS consensus group to encompass the terms hot comb alopecia, follicular degeneration syndrome, pseudopelade in African Americans, and central elliptical pseudopelade in Caucasians.In general, it is a condition that presents with flesh-colored, noninflammatory cicatricial alopecia of the central scalp that, over time, enlarges centrifugally. Pseudopelade of Brocq can mimic alopecia areata, lichen planopilaris, and DLE. In addition, central centrifugal cicatricial alopecia(CCCA), a noninflammatory condition of the central scalp that is seen primarily in black women. CCCA usually presents as a symmetric patch, unlike the irregularly bordered, typically atrophic plaques seen in classic pseudopelade of Brocq Central Centrifugal Cicatricial Alopecia (CCCA) is a form of scarring alopecia on the scalp that results in permanent hair loss. It is the most common form of scarring hair loss seen in black women. However, it may be seen in men and among persons of all races and hair color (though rarely). Middle-aged women are most commonly affected. Early diagnosis of CCCA is important because medical intervention can prevent further progression that often results in extensive, permanent hair loss. Diagnosis is based on clinical features, scalp biopsy and exclusion of other hair loss disorders. Scalp biopsy should be taken from an active edge of a patch of alopecia rather than the centre of a scarred area. Histopathology reveals a lymphocytic inflammatory infiltrate (inflammatory cells) around the infundibulum (base of the hair follicle), and fibrosis (scarring). Premature desquamation (peeling) of the inner root sheath is a common feature.

Question 88

88. Yellow nail syndrome is associated with which of the following? A. Multiple myeloma B. Panhypopituitarism C. Diabetes mellitus D. Dermatophyte infection E. Lymphedema and bronchiectasis

Answer 88

Correct choice: E. Lymphedema and bronchiectasis Explanation: Lymphedema and bronchiectasis are associated with yellow nail syndrome.

Question 89

89. This hair finding is caused by mutations in what gene? A. SPINK 5 B. NEMO C. SERCA2 D. Dyskerin E. Keratin 16

Answer 89

Correct choice: A. SPINK 5 Explanation: Mutations in SPINK 5, a serine protease inhibitor leads to Netherton’s syndrome which is characterized by trichorrhexis invaginata or bamboo hair. Light microscopic examination of clipped hair from the scalp and (of particularly high yield) the eyebrows often reveals trichorrhexis invaginata (bamboo hair) with a ball-and-socket appearance In incontinentia pigmenti, lack of NEMO (NF-κB essential modulator; also known as IKBKG [inhibitor of κ light polypeptide gene enhancer in B cells, kinase γ]) results in failure to activate NF-κB, which normally protects against tumor necrosis factor (TNF)-α-induced apoptosis. Darier disease is due to mutations in ATP2A2 which encodes SERCA2. Dyskerin interacts with telomerase and plays a role in its proper function. Keratin 16 is a defect seen in pachyonychia congenita.

Question 90

90. Which part of the hair follicle is the first to cornify? A. Huxley’s layer of inner root sheath B. Henley’s layer of inner root sheath C. Outer root sheath D. Medulla E. Cuticle

Answer 90

Correct choice: B. Henley’s layer of inner root sheath Explanation: Henley’s layer of inner root sheath. Henleys layer is the outer layer of the inner root sheath. The inner root sheath is keratinized with trichohyaline granules and is shed with the growing hair shaft at the level of the isthmus. The remaining choices are incorrect.

Question 91

91. A 24-year-old man reports that he has developed white patches of scalp hair that seem to migrate across his scalp. Examination reveals three 1 cm foci of poliosis with surrounding normally pigmented hair. He has a history of hypothyroidism, but no other systemic diseases. What is the most likely etiology: A. Form fruste of alopecia areata B. Vogt-Koyanagi-Harada syndrome C. Tinea capitis D. Alezzandrini syndrome E. Lichen planopilaris

Answer 91

Correct choice: A. Form fruste of alopecia areata Explanation: Forme fruste, or limited, alopecia areata, may present with migratory poliosis due to destruction of follicular melanocytes but survival of the hair-producing bulb. Vogt-Koyanagi-Harada syndrome presents as meningoencephalitis followed by visual and hearing deficits and poliosis of the periorbital region.

Question 92

92. Which of the following factors is most strongly associated with a negative prognosis in the condition pictured? A. Onset in adulthood B. Atopic dermatitis C. Short duration of disease D. Limited extent of disease E. Absent nail changes

Answer 92

Correct choice: B. Atopic dermatitis Explanation: Atopic dermatitis is a negative prognostic factor in alopecia areata (AA). This question asks the examinee to recognize AA by a solitary round patch of non-scarring scalp alopecia, and determine that of the answer choices, only atopic dermatitis (choice 2) carries a negative prognosis. Other negative prognostic factors in AA include childhood onset (not adult onset, choice 1), prolonged disease duration (not short duration, choice 3), diffuse extent of disease (not limited extent, choice 4), and presence of nail dystrophy (not absence, choice 5).

Question 93

93. A 68-year old man presents with double white transverse lines on all nails that disappear with compression of the nail plate. What is the most likely associated systemic disease: A. Ehlers-Danlos syndrome B. Arsenic poisoning C. Rheumatic fever D. Neurofibromatosis E. Nephrotic syndrome

Answer 93

Correct choice: E. Nephrotic syndrome Explanation: Muehrcke’s lines are white transverse lines from an abnormal vascular bed. They disappear with pressure on the nail plate. Muehrcke’s lines are associated with disorders causing low albumin, such as nephrotic syndrome, liver disease, and malnutrition. The alternative choices are incorrect.

Question 94

94. Trichobezoar is a potentially life-threatening complication of: A. Alopecia areata B. Anhidrotic ectodermal dysplasia C. Trichotillomania D. Discoid lupus E. Lichen planopilaris

Answer 94

Correct choice: C. Trichotillomania Explanation: Trichophagia is more common in individuals who have trichotillomania. This chewing behavior frequently can lead to the formation of trichobezoars in the stomach or small intestines. Trichobezoars can result in anemia, abdominal pain, hematemesis, nausea and/or vomiting, bowel obstruction, perforation, gastrointestinal (GI) bleeding, acute pancreatitis, and obstructive jaundice. The other answer choices are not associated with trichobezoars.

Question 95

95. What determines the thickness of the nail plate? A. Hyponychium B. Nail matrix C. Nail bed D. Proximal nail fold E. Nail plate

Answer 95

Correct choice: B. Nail matrix Explanation: The nail matrix determines the thickness of the nail plate. The matrix produces cells that become the nail plate. The width and thickness of the nail plate is determined by the size, length, and thickness of the matrix, while the shape of the fingertip itself shows if the nail plate is flat, arched, or hooked. The hyponychium is the area of epithelium, particularly the thickened portion, underlying the free edge of the nail plate on the nail. The nail bed is the skin beneath the nail plate. The skin proximal to the nail that covers the nail foldis the eponychium. The tissue distal to the eponychium in contact with the nail represents the cuticle. Extending from proximal to distal on the nailis a half-moon shaped white arc known as the lunula (see the image below). Nail Plate: It is the actual fingernail that is made of translucent keratin protein composed of amino acids. It is produced by a type of modified epidermis. The plate is the actual nail.

Question 96

96. A thick scale with matted hair is seen on a patient's head. The most likely diagnosis is: A. Pityriasis amiantacea B. Traction alopecia C. Triangular alopecia D. Loose anagen syndrome E. Telogen effluvium

Answer 96

Correct choice: A. Pityriasis amiantacea Explanation: Pityriasis amiantacea is a condition seen in pediatric patients that improves with age. There is thick scale and matted hair that may mimic seborrheic dermatitis or psoriasis. Treatment is keratolytics. The other options would not typically present with scale.

Question 97

97. A 32 year-old woman presents with moderate hirsutism. She has normal menses, normal-sized ovaries, no evidence of tumors of the adrenal or ovary, and normal adrenal function, but does have slight elevations of plasma androstenedione and testosterone. What is the most likely diagnosis? A. Stein-Leventhal syndrome B. Cushing syndrome C. Idiopathic hirsutism D. Occult virilizing tumor E. Kruckenberg tumor

Answer 97

Correct choice: C. Idiopathic hirsutism Explanation: Idiopathic hirsutism is diagnosed in women with evidence of androgen excess but with normal menses, normal-sized ovaries, no evidence of tumors of the adrenal or ovary, and normal androgen function. Slight elevations of plasma androstenedione and testosterone are common. The other answer choices listed are incorrect.

Question 98

98. Which of the following syndromes does not have Dorsal pterygium as a feature: A. Lichen planus B. Lesch-Nyhan syndrome C. Cicatricial pemphigoid D. Chronic GVHD E. Lamellar ichthyosis

Answer 98

Correct choice: E. Lamellar ichthyosis Explanation: Dorsal pterygium (scarring of the proximal nail fold) is caused by lichen planus, acrosclerosis, onychotillomania, Lesch-Nyhan syndrome, chronic GVHD, SJS/TEN and cicatricial pemphigoid.All of the listed items are correct except lamellar ichthyosis. It does not have this nail finding.

Question 99

99. This describes a persistent patch of vellus hairs or complete hair loss in the frontal-temporal region. This condition is called: A. Triangular alopecia B. Loose anagen syndrome C. Telogen effluvium D. Anagen effluvium E. Trichotillomania

Answer 99

Correct choice: A. Triangular alopecia Explanation: Triangular alopecia is also known as temporal alopecia and describes a persistent triangular patch of vellus hairs or complete hair loss in the frontal-temporal region. On histology, there is vellus hairs seen. There is usually no treatment for this condition. Loose anagen syndrome presents as loss of clumps of hair. A hair pull test would show anagen hairs with ruffled cuticles. Telogen effluvium occurs 2-3 months after an inciting stressful event such as nutritional issues, endocrine changes (thyroid disorders, post-partum), or certain medications. Anagen effluvium presents as a sudden loss of anagen hairs typically occuring in the setting of radiation, chemotherapy, mercury poisoning, severe protein malnutrition, or thallium use. Trichotillomania presents as compulsive hair pulling with irregular broken hairs within a geometric localized area.

Question 100

100. Lindsay's nails are associated with which of the following disorders? A. Cirrhosis B. Wilson's disease C. Chronic bronchiectasis D. Chronic renal failure E. Arsenic poisoning

Answer 100

Correct choice: D. Chronic renal failure Explanation: Lindsay's nails (also called half-and-half nails) refers to the proximal half of the nail plate appearing white, and the distal half of the nail plate containing a red-brown zone. It is classically associated with chronic renal failure. Cirrhosis is associated with Terry's nails. Blue lunulae are associated with Wilson's disease. Yellow nails are associated with chronic bronchiectasis. Arsenic poisoning is associated with red lunulae.

Question 101

101. This is a small reddish-blue tender subungual tumor that presents with the following histology: A. Glomus tumor B. Myxoid cyst C. Acquired digital fibrokeratoma D. Accessory digit E. Verruca Vulgaris

Answer 101

Correct choice: A. Glomus tumor Explanation: A glomus tumor is a small reddish-blue tender subungual tumor. On pathology, one can see small uniform round cells with eosinophilic cytoplasm. A myxoid cyst presents as a soft, smooth nodule most commonly adjacent to the DIP joint. When at the proximal nail fold, it can cause longitudinal grooving in the nail plate. On pathology, there is a large pool of mucin with no lining. An acquired digital fibrokeratoma presents as a firm nodule on the finger or toe. Pathology presents as collagen with no prominent nerves. An accessory digit also presents as a firm nodule on the finger or toe, most commonly at the proximal portion of the fifth digit. Pathology presents as collagen with prominent nerve fascicles. Verruca Vulgaris presents as well defined hyperkeratotic papules around the nails. Pathology presents with acanthosis, papillomatosis, and hyperkeratosis.

Question 102

102. Which of the following is NOT a cause of red lunulae? A. Endocarditis B. Psoriasis C. Carbon monoxide poisoning D. Azathioprine E. Rheumatoid arthritis

Answer 102

Correct choice: A. Endocarditis Explanation: Endocarditis is not a cause of red lunulae. Alopecia areata, psoriasis, prednisone for connective tissue diseases, carbon monoxide poisoning, cardiac failure, systemic lupus erythematosus, rheumatoid arthritis, chronic obstructive pulmonary disease, cirrhosis, azathioprine and radiation are the causes of this condition.

Question 103

103. Proximal white subungual onychomycosis with polydactylous involvement needs at least one laboratory test: A. Blood count B. Sedimentation rate C. T-helper CD4 lymphocytes D. Liver enzymes E. HIV

Answer 103

Correct choice: E. HIV Explanation: This type of onychomycosis has a prognostic value: It means that the CD4 lymphocytes are below 450. The other lab tests are not as important as checking HIV status. c

Question 104

104. Patients that have been diagnosed with hypohidrotic ectodermal dysplasia have the hair findings of: A. Longitudinal groove on electron microscopy B. Trichorrhexis nodosa C. Monilethrix D. Trichorrhexis invaginata E. Pili trianguli et canaliculi

Answer 104

Correct choice: A. Longitudinal groove on electron microscopy Explanation: Patients with hypohidrotic ectodermal dysplasia have been found to possess hairs with longitudinal grooves on electron microscopy. It is important to note that these hairs do NOT have a triangular cross-section, thereby distinguishing them from hairs with the finding of pili trianguli et canaliculi. Trichorrhexis nodosa refers to an incomplete fracture with frayed ends resembling two paint brushes against each other (seen in Menkes disease, trichothiodystrophy, arginosuccinic aciduria, and Netherton's syndrome). Monilethrix refers to the beaded appearance of hair due to periodic thinning of the hair shaft. Trichorrhexis invaginata, also called "bamboo hair," is the term used to describe the microscopic appearance of the hair similar to a "ball and socket" or "collapsible telescope." It is seen in Netherton's syndrome. Pili trianguli et canaliculi (aka "spun glass hair") refers to a disorder of the hair in which there is premature keratinization of the inner root sheath, resulting in hairs with a triangular cross-section and a central linear groove along one side. This is seen in the uncombable hair syndrome. c

Question 105

105. Telogen hairs are in the resting phase, which lasts for: A. 3 months B. 1 month C. 2 months D. 4 months E. 5 months

Answer 105

Correct choice: A. 3 months Explanation: Telogen hairs are at the resting phase, make up 10-15% of hairs, and last for 3 months. Anagen phase is the growing phase and lasts for 2-6 years. Catagen hairs are in the transition phase, which lasts for 2-3 weeks.

Question 106

106. Familial alopecia areata is commonly associated with what laboratory abnormality? A. Hypocalcemia B. Hypereosinophilia C. Hyperkalemia D. Leukopenia E. Thrombocytopenia

Answer 106

Correct choice: E. Thrombocytopenia Explanation: Familial alopecia areata has been associated with persistent thrombocytopenia. Alopecia areata has recently been linked to the ULBP3 gene. Alopecia areata is associated with other autoimmune disorders such as thyroid disease and vitiligo.

Question 107

107. What are the nail findings in this condition? A. Red-white longitudinal bands and v-shaped distal nicks B. Oil spots and onycholysis C. Clubbing D. Dolichonychia E. Trachyonychia

Answer 107

Correct choice: A. Red-white longitudinal bands and v-shaped distal nicks Explanation: Darier’s disease presents with hyperkeratotic papules in seborrheic areas, acrokeratosis verruciformis of Hopf, palmar keratosis and pits, and nail findings including red-white longitudinal bands and v-shaped distal nicks. Oil spots and onycholysis may occur in psoriasis. Clubbing may occur in a variety of conditions such as Hypertrophic pulmonary osteoarthropathy. Dolichonychia may occur in Ehlers-Danlos and Marfan’s syndrome. Trachyonychia may occur in lichen planus.

Question 108

108. A 14-year-old man with a history of extensive striae, upward lens dislocation, and pectus excavatum would be most likely to present with what nail abnormality: A. Beau’s lines B. Mee’s lines C. Muehrcke’s lines D. Onychophagia E. Dolichonychia

Answer 108

Correct choice: E. Dolichonychia Explanation: Dolichonychia exhibits long, slender nails, and is associated with Ehlers-Danlos and Marfans. Beau’s lines are transverse indented nail plate furrows caused by temporary growth arrest of the nail matrix, often due to chemotherapy or other stressful events and illnesses. Mee’s lines are transverse white lines affecting all nails and growing out with the nail; there is no associated indentation. Mee’s lines may be caused by arsenic poisoning, rheumatic fever, or other systemic diseases. Muehrcke’s lines disappear with squeezing of the nail and are due to disorders of low albumin. Oncyhophagia refers to nail biting.

Question 109

109. Scalp biopsy of which of the following types of hair loss will reveal increased telogen hairs? A. Lichen planopilaris B. Discoid lupus erythematosis C. Central centrifugal alopecia D. Dissecting cellulitis E. Androgenetic alopecia

Answer 109

Correct choice: E. Androgenetic alopecia Explanation: Androgenetic alopecia, telogen effluvium, and alopecia areata all have an increased fraction of telogen hairs. Lichen planopilaris and discoid lupus typically reveal a chronic inflammatory alopecia on biopsy. Dissecting cellulitis typically exhibits chronic inflammation with hypertrophic scar tissue intervening between suppurative follicles

Question 110

110. A patient presents with a focal absence of epidermis on the scalp. This can be associated with: A. Adams-Oliver syndrome. B. Parry Romberg syndrome C. Cicatricial pemiphigoid D. Mastocytosis E. Epidermal nevi

Answer 110

Correct choice: A. Adams-Oliver syndrome Explanation: Patients that are diagnosed with Adams-Oliver syndrome have aplasia cutis congenita (ACC) over the posterior scalp, terminal transverse defects of the limbs, and small toenails. Most aplasia cutis congenita cases are on the scalp and present as single lesions. ACC can also be associated with Trisomy 13.

Question 111

111. Cutaneous signs of cortisol excess include all of the following EXCEPT: A. Plethora B. Atrophic/fragile skin C. Striae distensae D. Dorsocervical/Supraclavicular fat pads E. Peripheral obesity

Answer 111

Correct choice: E. Peripheral obesity Explanation: All of the listed options are signs of cortisol excess except peripheral obesity. The most common pattern of obesity in patients with cortisol excess is centripetal or central obesity.

Question 112

112. Thin hair with premature graying is characteristic of which of the following syndromes? A. Werner syndrome B. Turner Syndrome C. Noonan Syndrome D. Cornelia de Lange Syndrome E. Waardenburg's syndrome

Answer 112

Correct choice: A. Werner syndrome Explanation: Werner syndrome is characterized by premature degeneration of major organs that loosely mimics premature aging. It is associated with sclerodermoid changes, leg ulcerations, premature graying, cataracts, premature atherosclerosis,a dn diabetes. It is associated with defects in the helicase RecQL2. Thin hair with premature graying is not characteristic of the remaining listed syndromes.

Question 113

113. Patients that have a normal distal nail and a proximal 1/2 nail that is white is associated with: A. Chronic renal failure B. Pulmonary stenosis C. Gastrointestinal malignancy D. Tuberous sclerosis E. Cirrhosis

Answer 113

Correct choice: A. Chronic renal failure Explanation: Patients that have a normal distal nail and a proximal white nail have a condition called Lindsay's nails or half and half nail. The proximal nail that is white is from nail bed edema. This is from chronic renal failure.

Question 114

114. During a routine physical examination, you notice fusion of the hyponychium to the distal nail plate on some of your patient's fingers. Which of the following conditions may produce these nail findings? A. Lichen planus B. Cicatricial pemphigoid C. Graft-versus-host disease D. Systemic sclerosis E. Marfan syndrome

Answer 114

Correct choice: D. Systemic sclerosis Explanation: The nail finding described in the question stem is that of ventral pterygium (pterygium inversum unguis), which can be familial or secondary to trauma, systemic lupus erythematosus, or systemic sclerosis. Lichen planus, cicatricial pemphigoid, and GVHD may produce dorsal pterygium, which is described as a wing-like growth fusing the proximal nail fold with the nail bed/ matrix. Marfan syndrome is associated with dolichonychia (long nails).

Question 115

115. Which of the following medications is a cause of hirsutism without virilization? A. Dapsone B. Diazoxide C. Diazepam D. Dinitrochlorobenzene E. Dantrolene

Answer 115

Correct choice: B. Diazoxide Explanation: Phenytoin, diazoxide, cyclosporine and hexachlorobenzene all can cause increased hair growth in patients. In addition, oral (and topical) minoxidil can cause hirsutism. The other listed medications do not cause hirsutism.

Question 116

116. A 44-year-old man undergoing chemotherapy for Hodgkin’s lymphoma presents with horizontal rough bands involving the nail plates of all digits. What is the most likely disorder: A. Beau’s lines B. Mee’s lines C. Muehrcke’s lines D. Onychophagia E. Dolichonychia

Answer 116

Correct choice: A. Beau’s lines Explanation: Beau’s lines are transverse indented nail plate furrows caused by temporary growth arrest of the nail matrix, often due to chemotherapy or other stressful events and illnesses. Mee’s lines are transverse white lines affecting all nails and growing out with the nail; there is no associated indentation. Mee’s lines may be caused by arsenic poisoning, rheumatic fever, or other systemic diseases. Muehrcke’s lines disappear with squeezing of the nail and are due to disorders of low albumin. Oncyhophagia refers to nail biting. Dolichonychia exhibits long, slender nails, and is associated with Ehlers-Danlos and Marfans.

Question 117

117. Which of the following are characteristics of Trichorhinophalangeal syndrome? A. Kinky hair, frontal bossing, small widely spaced teeth with poor enamel B. Palmoplantar keratoderma, trichorrhexis nodosa, sinus infections C. Shortened phalanges, sparse hair, bulbous nose D. Brittle hair, short stature, cerebellar ataxia E. Sparse fine hair, Short extremities, Immunodeficiency

Answer 117

Correct choice: C. Shortened phalanges, sparse hair, bulbous nose Explanation: Characteristic features of trichorhinophalangeal syndrome are shortened phalanges, sparse hair, and bulbous nose. Trichorhinophalangeal syndrome type I (TRPS I) is a condition that causes bone and joint malformations; distinctive facial features; and abnormalities of the skin, hair, teeth, sweat glands, and nails. The name of the condition describes some of the areas of the body that are commonly affected: hair (tricho-), nose (rhino-), and fingers and toes (phalangeal). In people with TRPS I, the ends (epiphyses) of one or more bones in the fingers or toes are abnormally cone-shaped. Additionally, the fingernails and toenails are typically thin and abnormally formed. Affected individuals often have short feet. Individuals with TRPS I may have a misalignment of the hip joints (hip dysplasia), which often develops in early adulthood but can occur in infancy or childhood. Children with TRPS I often have an unusually large range of movement (hypermobility) in many of their joints. Over time, however, the joints may break down (degenerate), leading to joint pain and a limited range of joint movement. The characteristic appearance of individuals with TRPS I involves thick eyebrows; a broad nose with a rounded tip; large ears, a long, smooth area between the nose and the upper lip (philtrum); a thin upper lip; and small teeth that are either decreased (oligodontia) or increased(supernumerary) in number. Almost all affected individuals have sparse scalp hair. Males are particularly affected by hair loss with many being nearly or completely bald soon after puberty. Some children with this condition have loose skin, but the skin becomes tighter over time. Individuals with TRPS I may experience excessive sweating (hyperhidrosis).

Question 118

118. Distal subungual onychomycosis associated with paronychia is due to: A. Candida spp B. Fusarium spp C. Scytalidium dimidiatum D. Trichophyton rubrum E. Trichophyton mentagrophytes

Answer 118

Correct choice: C. Scytalidium dimidiatum Explanation: This mold is the only one responsible for paronychia in DLSO and is more often observed in finger than in toenails.

Question 119

119. Which of the following is a manifestation of psoriasis of the nail matrix? A. Splinter hemorrhages B. “Oil spots” C. Subungual hyperkeratosis D. Pits E. Onycholysis

Answer 119

Correct choice: D. Pits Explanation: Psoriatic nail changes may be of nail matrix or nail bed origin. Pits are the common finding; splinter hemorrhages the least. Psoriatic nail changes of matrix origin include: pits (representing focal psoriasis of the proximal matrix) and leukonychia. Psoriatic nails changes of nail bed origin include: salmon spots, “oil spots,” onycholysis, subungual hyperkeratosis, and splinter hemorrhages.

Question 120

120. A rapid onset of hair growth with or without accompanying virilization can occur in all of the following conditions EXCEPT: A. Adrenal adenoma B. Adrenal carcinoma C. Arrhenoblastoma D. Krukenburg tumor of the ovary E. Ovarian cyst

Answer 120

Correct choice: E. Ovarian cyst Explanation: An ovarian cyst does not cause a rapid onset of hair growth with or without accompanying virilization, as it does not produce androgens. Women with polycystic ovarian syndrome (PCOS) may have signs of hyperandrogenism, but this is not due to the ovarian cysts per se. All of the other options can cause a rapid onset of hair growth with or without accompanying virilization. An Arrhenoblastoma is a tumor of the ovary that secretes testosterone.

Question 121

121. A patient with diffuse severe sudden hair loss developing 3 months after hospitalization for septicemia likely has which of the following conditions? A. Anagen effluvium B. Catagen effluvium C. Telogen effluvium D. Loose anagen syndrome E. Uncombable hair syndrome

Answer 121

Correct choice: C. Telogen effluvium Explanation: Telogen effluvium typically ensues stressful events after a 2-3 month period. The other answer choices do not likely represent this clinical scenario.

Question 122

122. Which of the following conditions with hair abnormalities is caused by mutations in a DNA helicases? A. Anhidrotic Ectodermal Dysplasia B. Hidrotic Ectodermal Dysplasia C. KID Syndrome D. Rothmund-Thomson Syndrome E. Incontinentia Pigmenti

Answer 122

Correct choice: D. Rothmund-Thomson Syndrome Explanation: Rothmund-Thopmson syndrome is casued by mutations in RecQL4. Key clinical features include poikiloderma, photosensitivity, dystrophic nails, hypoplastic thumbs, cataracts, and reports of osteosarcoma. Anhidrotic ectodermal dysplasia is associated with mutations in ectodysplasin. Hidrotic ectodermal dysplasia is associated with mutations in connexin 30. Kid syndrome is associated with mutations in connexin 26 and IP with NEMO.

Question 123

123. All of the following are associated with anonychia or absence of the nail except: A. Nail-patella syndrome B. Congenital onychodysplasia of the index finger C. Hidrotic Ectodermal Dysplasia D. Lichen Planus E. Ehlers-Danlos syndrome

Answer 123

Correct choice: E. Ehlers-Danlos syndrome Explanation: All of the following, except Proteus syndrome, is associated with anonychia or absence of the nail. Ehlers-Danlos syndrome is associated with Dolichonychia, i.e. long nails with a length to width ratio > 1.

Question 124

124. What nutritional deficiency can lead to this nail finding? A. Vitamin B12 B. Vitamin C C. Iron D. Magnesium E. Thiamine

Answer 124

Correct choice: C. Iron Explanation: Iron deficiency can lead to spoon nail deformity or koilonychias. Vitamin B12 deficiency results in glossitis and hyperpigmentation. Vitamin C deficiency leads to epistaxis, poor wound healing, and hemorrhagic gingivitis.

Question 125

125. This is a term used to describe the thin epithelium immediately beneath the nail plate, with an absent granular layer: A. Nail bed B. Hyponychium C. Nail matrix D. Proximal nail fold E. Nail plate

Answer 125

Correct choice: A. Nail bed Explanation: The nail bed is described as the thin epithelium immediate beneath the nail plate. It does not have a granular layer. The hyponychium describes the epithelium that lies on the volar surface of the digit. The nail matrix is the epithelium which starts at the mid distal phalanx. It generates the nail plate and keratinizes without a granular layer. The proximal nail fold is the dorsal part of the digit that lies adjacent to the nail. The nail plate is the actual nail made up of tightly packed onychocytes.

Question 126

126. This is a term used to describe the transverse excessive curvature from the widened base of the terminal phalanx. It is called: A. Pincer nails B. Platyonychia C. Plicatured nails D. Plummer's nails E. Dorsal pterygium

Answer 126

Correct choice: A. Pincer nails Explanation: Transverse excessive curvature from the widened base of the terminal phalanx is called pincer nails. It can be hereditary or from shoes. It usually involves the first toenails. It is associated with subungual exostoses, osteoarthritis, and psoriasis. Platyonychia describes nails with increased curvature in the long axis. Plicatured nails are flat transversely and have vertical lateral drops in the nail. Plummer's nails are onycholysis occuring from hypothyroidism. Dorsal pterygium describes scarring of the proximal nail fold occuring in lichen planus, acrosclerosis, and Lesch-Nyhan syndrome.

Question 127

127. Which of the following may produce red lunulae? A. Penicillamine B. Darier disease C. Nail-Patella syndrome D. Lupus erythematosus E. Arsenic poisoning

Answer 127

Correct choice: D. Lupus erythematosus Explanation: Red lunulae may be associated with the following: alopecia areata, rheumatoid arthritis, lupus erythematosus (both systemic and cutaneous), congestive heart failure, and carbon monoxide poisoning. Penicillamine may produce yellow nails. Darier disease is associated with longitudinal alternating red-and-white bands as well as V-shaped distal nicking. Triangular lunulae are found in Nail-Patella syndrome. Arsenic poisoning is associated with Mee's lines, which are transverse lines of entire nail breadth in all nails.

Question 128

128. Deposition of mucin in the hair follicle can be associated with which disease? A. Keratosis Follicularis Spinulosa Decalvans B. Adams-Oliver Syndrome C. Mycosis fungoides D. Acne Keloidalis Nuchae E. Perifolliculitis Capitis Abscedens et Suffodiens

Answer 128

Correct choice: C. Mycosis fungoides Explanation: Mycosis fungoides can be associated with follicular mucinosis. Follicular mucinosis is not reported to be associated with the other listed conditions.

Question 129

129. This is used to describe a follicular degeneration syndrome mainly on the crown of the scalp, also known as hot-comb alopecia: A. Central centrifugal cicatricial alopecia B. Alopecia mucinosa C. Traction alopecia D. Pseudopelade of Brocq E. Lichen planopilaris

Answer 129

Correct choice: A. Central centrifugal cicatricial alopecia Explanation: Central centrifugal cicatricial alopecia is a follicular degeneration syndrome with loss mainly on the crown of the scalp and occurs secondary to tight braids, ponytails, hair straighteners/ curlers, or chemical relaxers. Pathology shows premature desquamation of the inner root sheath with fibrosis. Alopecia mucinosa presents as alopecia or broken hairs on the scalp or beard. It typically occurs primary or secondary to CTCL. Traction alopecia is hair loss secondary to tight braids or hair styles with traction. It typically presents on the frontal and parietal scalp. Pseudopelade of Brocq is an end-stage scarring alopecia presenting as "footprints in the snow". Lichen planopilaris is alopecia associated with perifollicular erythema.

Question 130

130. A patient has hyperkeratotic tissue on the lateral and proximal nails folds. What is the diagnosis? A. Onychophosis B. Onychomadesis C. Hapalonychia D. Onychocryptosis E. Onychophagia

Answer 130

Correct choice: A. Onychophosis Explanation: Onychophosis describes hyperkeratosis tissue on the lateral and proximal nails folds. Onychomadesis is shedding of the nail starting at the proximal end. Hapalonychia refers to soft, bendable nails. Onychocryptosis refers to an ingrown toenails. Onychophagia means nail biting.

Question 131

131. The most common cause for superficial white onychomycosis is: A. Trichophyton rubrum B. Trichophyton mentagrophytes C. Epidermophyton floccosum D. Fusarium oxysporum E. Scopulariopsis brevicalis

Answer 131

Correct choice: B. Trichophyton mentagrophytes Explanation: T. mentagrophytes is the most common cause of superficial white onychomycosis. Other causes include: Fusarium, Acremonium and Aspergillus spp. The other options are not the most common cause of superficial white onychomycosis.

Question 132

132. A 26-year-old woman presents with hair loss. Examination of a single shaft reveals division into two shafts in short segments, with each individually covered with a complete cuticle. What is the most likely diagnosis: A. Pili multigemini B. Pili bifurcati C. Trichoptilosis D. Trichoschisis E. Trichothiodystrophy

Answer 132

Correct choice: B. Pili bifurcate Explanation: Pili bifucarti exhibits hair shaft bifurcation into short segments along hair shafts, with each segment covered with it’s own cuticle. Pili multigemini refers to a rare disorder of multiple divided hair matrices, with multiple hair shafts each with their own cuticle all arising from one follicle. Trichoptilosis refers to “split ends” of the distal portion of the hair shaft. Trichoschisis is a clean fracture of the hair shaft. Trichothiodystrophy is a disorder of beaded hair when viewed with polarizing light.

Question 133

133. The combination of poliosis, uveitis, deafness, and vitiligo are characteristic of which of the following conditions? A. Piebaldism B. Waardernburg syndrome C. Cronkhite-Canada syndrome D. Vogt-Koyanagi-Harada syndrome E. Cornelia de Lange Syndrome

Answer 133

Correct choice: D. Vogt-Koyanagi-Harada syndrome Explanation: Vogt-Koyanagi-Harada syndrome is a presumed autoimmune condition characterized by poliosis, chronic uveitis, deafness, and vitiligo. The other conditions do not typically present with this combination of symptoms.

Question 134

134. The "ragged cuticle" seen in dermatomyositis is also known as: A. Samitz sign B. Ventral pterygium C. Plummers sign D. Candy-cane nails E. Emperipolesis

Answer 134

Correct choice: A. Samitz sign Explanation: Cuticular fraying from dermatomyositis is called the Samitz sign or "ragged cuticle". The other options are not associated with dermatomyositis. A ventral pterygium can be seen in systemic sclerosis. There is no such sign as Plummers sign. Candy-cane nails are seen in Darier's disease. Emperipolesis is a histological finding seen in Rosai-Dorfman disease and is not a nail finding.

Question 135

135. Psoriatic onycholysis is caused by psoriasis in which of the following nail subunits? A. Nail matrix B. Nail plate C. Nail bed D. Proximal nail fold E. Hyponychium

Answer 135

Correct choice: C. Nail bed Explanation: Nail bed involvement by psoriasis often causes onycholysis, oil drops, dyschromia, splinter hemorrhages, or subungula hyperkeratosis. The other answer choices do not describe the correct site of psoriatic involvement causing onycholysis. Nail matrix psoriasis demonstrates pitting, beau's lines, leukonychia, red spots in the lunula, or nail plate crumbling.

Question 136

136. Pili Annulati is associated with which of the following syndromes? A. Netherton B. PIBIDS C. Citrullinemia D. Hidrotic ectodermal dysplasia E. Alopecia areata

Answer 136

Correct choice: E. Alopecia areata Explanation: Pili Annulati is the alternating light and dark bands secondary to air-spaces seen in normal light and has been associated with Alopecia areata. The "tiger-tail" pattern seen in trichothiodystrophy (PIBIDS) is only seen with polarized light. Netherton and Citrullinemia do not have this hair finding associated. Pili annulati or ringed hair is used to describe hair in which there are scattered groups of air-filled 'bubbles' along the hair shaft. These appear as bright bands when seen with reflected light.

Question 137

137. This condition is used to describe an impulse control disorder with the repeated plucking or pulling of hairs: A. Trichotillomania B. Anagen effluvium C. Telogen effluvium D. Loose anagen syndrome E. Triangular alopecia

Answer 137

Correct choice: A. Trichotillomania Explanation: Patients with trichotillomania have an impulse control disorder with repeated plucking or pulling of hair. This presents as a confluence of irregular, short sparse hairs within an otherwise normal area of the scalp. On pathology, there would be increased catagen hairs, trichomalacia, and melanin in the follicular canal. Anagen effluvium presents as a sudden loss of anagen hairs typically occuring in the setting of radiation, chemotherapy, mercury poisoning, severe protein malnutrition, or thallium use. Telogen effluvium occurs 2-3 months after an inciting stressful event such as nutritional issues, endocrine changes (thyroid disorders, post-partum), or certain medications. Loose anagen syndrome presents as loss of clumps of hair. A hair pull test would show anagen hairs with ruffled cuticles. Triangular alopecia presents as a complete absence of hair or vellus hairs in a triangular pattern in the temporal area, frequently bilateral.

Question 138

138. Arsenical poisoning is associated with what nail finding? A. Muehrcke's lines B. Mee's lines C. Hapalonychia D. Onycholysis E. Oil spots

Answer 138

Correct choice: B. Mee's lines Explanation: Arsenical poisoning is characteristically associated with Mee's lines.

Question 139

139. A patient presents with silvery, blond, and gray hair over the entire body at birth or early infancy. The acquired form of this condition is associated with: A. Fetal alcohol syndrome B. Ambras syndrome C. Universal Hypertrichosis D. Congenital adrenal hyperplasia E. Arrhenoblastoma

Answer 139

Correct choice: A. Fetal alcohol syndrome Explanation: Patients with generalized congenital hypertrichosis lanuginosa present with silvery, blond, and gray hair over the entire body at birth or early infancy. It is an autosomal dominant condition with dental anomalies. The acquired form is associated with fetal hydantoin and fetal alcohol syndrome. Ambras syndrome presents with thicker, longer hair increased on the face, ears, and shoulders. There is also associated facial dysmorphism and dental anomalies. Universal hypertrichosis presents with a "wolf boy" phenotype with thick hair everywhere. Congenital adrenal hyperplasia most commonly occurs from a 21-hydroxylase mutation and presents with precocious puberty, hirsutism, and early androgenetic alopecia. Arrhenoblastoma is a tumor causing the rapid onset of hair growth with or without accompanying virilization.

Question 140

140. A 16-year-old woman presents with hair breakage. Examination of a single shaft reveals division into two shafts at the distal-most portion of the hair. What is the most likely diagnosis: A. Pili multigemini B. Pili bifurcati C. Trichoptilosis D. Trichoschisis E. Trichothiodystrophy

Answer 140

Correct choice: C. Trichoptilosis Explanation: Trichoptilosis refers to “split ends” of the distal portion of the hair shaft. Trichoschisis is a clean fracture of the hair shaft. Trichothiodystrophy is a disorder of beaded hair when viewed with polarizing light. Pili multigemini refers to a rare disorder of multiple divided hair matrices, with multiple hair shafts each with their own cuticle all arising from one follicle. Pili bifucarti exhibits hair shaft bifurcation into short segments along hair shafts, with each segment covered with it’s own cuticle.

Question 141

141. Congenital generalized hypertrichosis is associated with which of the following symptoms? A. Androgen-secreting ovarian tumors B. Pituitary insufficiency C. Distichiasis D. Methimazole teratogenicity E. Gingival fibromatosis

Answer 141

Correct choice: E. Gingival fibromatosis Explanation: Congenital Generalized Hypertrichosis with Gingival Fibromatosis is a rare genetic condition with hypertrichosis on the face and upper body and gingival hyperplasia and fibromatosis. The other listed symptoms are not associated with congenital generalized hypertrichosis.

Question 142

142. Catagen hairs make up for less than 1% of the hair. This phase lasts for: A. 2-3 weeks B. 4-6 weeks C. 8-10 weeks D. 10-12 weeks E. 1 week

Answer 142

Correct choice: A. 2-3 weeks Explanation: Catagen phase ("transition phase") lasts up to 2-3 weeks and represents the transition from anagen to telogen. It makes up less than 1% of the hair population. The Anagen phase (growth phase) typically lasts 2-6 years. 85-90% of hairs are in this phase at any given time. The Telogen phase (resting phase) typically lasts 3 months. 10-15% of hairs are in this phase at any given time.

Question 143

143. Transverse white bands on one or two nails is caused by: A. Trauma to the matrix B. Alopecia areata C. Psoriasis D. Systemic lupus erythematosis E. Lichen planus

Answer 143

Correct choice: A. Trauma to the matrix Explanation: Longitudinal leukonychia or transverse white bands are caused by trauma to the nail matrix. Androgenetic alopecia, psoriasis, systemic lupus erythematosis and lichen planus all cause spotted red lunulae, not transverse white bands.

Question 144

144. Scarring of the proximal nail fold is called dorsal pterygium and is associated with all of the following except: A. Lichen planus B. Acrosclerosis C. Onychotillomania D. Lesch-Nyhan syndrome E. Osteoarthritis

Answer 144

Correct choice: E. Osteoarthritis Explanation: Patients with dorsal pterygiums are associated with all of the following except osteoarthritis. It is defined as scarring of the proximal nail fold. Dorsal pterygiums can also occur in chronic GVHD, SJS/TEN, and cicatricial pemphigoid.

Question 145

146. Distal subungual onychomycosis is most often caused by: A. Trichophyton mentagrophytes B. Epidermophyton floccosum C. Trichophyton schoenleinii D. Trichophyton rubrum E. Trichophyton megninii

Answer 145

Correct choice: D. Trichophyton rubrum Explanation: Trichophyton rubrum is the most common organism involved in distal subungual onychomycosis. The most common form of tinea unguium is distal subungual onychomycosis, which can also be distal and lateral. Distal subungual onychomycosis may develop in the toenails, fingernails or both. Some degree of tinea pedis is almost always present. The infection is usually caused byTrichophyton rubrum, which invades the nail bed and the underside of the nail plate, beginning at the hyponychium and then migrating proximally through the underlying nail matrix. Susceptibility to distal superficial onychomycosis may occur in an autosomal dominant pattern within families.

Question 146

147. An 84-year-old man presents to clinic with atypical fingernails and is diagnosed with hapalonychia. What defect does this term represent? A. Long nails B. White nail plates C. Edema of the nail bed D. Broad nails E. Soft nails

Answer 146

Correct choice: E. Soft nails Explanation: Hapalonychia refers to soft nails and is caused by a defect in the nail matrix. Dolichonychia describes abnormally long, narrow nails, while brachyonchia describes abnormally short, broad nails. Edema of the nail bed may present as Meurhcke’s lines.

Question 147

148. A 34-year-old man undergoing chemotherapy for acute myelogenous leukemia presents with smooth white horizontal bands involving the nail plates of all digits. What is the most likely disorder: A. Beau‟s lines B. Mee‟s lines C. Muehrcke‟s lines D. Onychophagia E. Dolichonychia

Answer 147

► B Beau‟s lines are transverse indented nail plate furrows caused by temporary growth arrest of the nail matrix, often due to chemotherapy or other stressful events and illnesses. Mee‟s lines are transverse white lines affecting all nails and growing out with the nail; there is no associated indentation. Mee‟s lines may be caused by chemotherapy, arsenic poisoning, rheumatic fever, or other systemic diseases. Muehrcke‟s lines disappear with squeezing of the nail and are due to disorders of low albumin. Oncyhophagia refers to nail biting. Dolichonychia exhibits long, slender nails, and is associated with Ehlers-Danlos and Marfans.

Question 148

_What is the common cause of the “one hand-two-foot” syndrome? A. Trichophyton rubrum B. Trichophyton mentagrophytes ( var. Interdigitale) C. Candida albicans D. Scytalidium dimidiatum E. Scopulariopsis brevicaulis

Answer 148

► A This condition is due to T. rubrum in 90% of the cases , T. interdigitale and Scytalidium dimidiatum share the 10% left.

Question 149

150- Mee's lines are: A. Double white transverse lines from abnormal vascular bed B. Brownish macules beneath the nail plate C. Local or diffuse hyperkeratotic tissue that develops on the lateral or proximal nail folds D. Transverse white lines that affect all nails, grow out with nail growth E. Vertical black lines on a single or multiple nails

Answer 149

► D Mee's lines are transverse white lines that affect all nails, grow out with nail growth. They are seen in arsenic poisoning, rheumatic fever, congenital heart failure, leprosy and with significant systemic disease. Brownish macules beneath the nail plate are oil spots, seen in psoriasis. Onychophosis is the local or diffuse hyperkeratotic tissue that develops on the lateral or proximal nail folds. The double white transverse lines from abnormal vascular bed are Muehrcke's lines which are caused by a nephrotic syndrome, low albumin, liver disease or malnutrition

Question 150

151- A patient with sparse hair, a pear-shaped broad nose and cone-shaped epiphyses likely suffers which of the following conditions? A. Focal Dermal Hypoplasia (Goltz syndrome) B. Tricho-rhino-phalangeal Syndrome C. Hay-Wells Syndrome D. Ectrodactyly-Ectodermal dysplasia-Clefting (EEC) E. Trichodentoosseous Syndrome

Answer 150

► B Tricho-rhino-phalangeal Syndrome is characterized by sparse hair, a pear-shaped broad nose and cone-shaped epiphyses.

Question 151

152- This hair disorder and mutations in ATP7A (MNK) gene are found in what genodermatosis? A. Bjornstad syndrome B. Menke's kinky hair syndrome C. Crandall's syndrome D. Bazex's follicular atrophoderma E. Trichothiodystrophy

Answer 151

► B Menke's kinky hair syndrome is characterized by pili torti and mutations in APT7A (MNK) gene. Bjornstad syndrome, Crandall‟s syndrome and Bazex‟s follicular atrophoderma are associated with pili torti but are not associated with this mutation.

Question 152

153- Which part of the matrix gives rise to the dorsal nail plate? A. Distal matrix B. Proximal matrix C. Ventral matrix D. Dorsal matrix E. Lateral matrix

Answer 152

► B The proximal matrix gives rise to the dorsal nail plate, while the distal matrix gives rise to the ventral nail plate.

Question 153

154- A 52-year-old man presents to clinic with erythematous papules and pustules in the region of the cheeks, chin, and neck that are worsened by shaving. Examination reveals pseudofolliculitis barbae. With what disease may this be associated: A. Cirrhosis B. Heart Failure C. Chronic Renal Failure D. Myeloma E. Emphysema

Answer 153

► C Pseudofolliculitis barbae has been associated with renal failure requiring hemodialysis, particularly in Caucasian patients. Most commonly, this syndrome arises in patients with darker skin types, whose hair shafts are elliptical in cross section, rather than round. This elliptical nature predisposes the hairs to kinking and invagination into the skin.

Question 154

155- A commonly known cause of splinter hemorrhages in the nail is endocarditis. Which of the following would be unlikely to cause splinter hemorrhages? A. Trichinosis B. Trauma C. Psoriasis D. Vasculitis E. Rheumatoid arthritis

Answer 154

► E Rheumatoid arthritis is not a cause of splinter hemorrhages of the nail. Endocarditis is the most commonly thought of cause of splinter hemorrhages. Trichinosis, trauma, psoriasis and vasculitis also can be causes of this also and should be considered when splinter hemorrhages are seen.

Question 155

156- Papular atrichia is caused by mutations in which gene? A. Wingless B. Patched C. Hairless D. Fox forehead E. Distal-less homebobox

Answer 155

► C Hairless gene mutations cause papular atrichia.

Question 156

157- Ventral pterygium is characteristically associated with what disorder? A. Lichen planus B. Systemic sclerosis C. Cicatricial pemphigoid D. SJS/TEN E. Chronic GVHD

Answer 156

► B Ventral pterygium is most characteristically associated with systemic sclerosis. All other choices are associated with dorsal pterygium.

Question 157

158- A patient is diagnosed having Menke's kinky's hair syndrome with a deficiency in: A. ATP7A gene B. ATP2A2 gene C. Argininosuccinic aciduria D. BCS1L gene E. ABCC2A gene

Answer 157

► A Patients with Menke's Kinky Hair syndrome have a X-linked recessive disorder in the ATP7A gene. This gene is responsible for the copper translocating membrane ATPase. They have brittle "steel wool" hair with a cupid's bow lips, pale and lax skin.

Question 158

159- Primary total dystrophic onychomycosis is due to: A. Candida spp B. Fusarium C. Trichophyton rubrum D. Scytalidium dimidiatum E. Scopulariopsis brevicaulis

Answer 158

► A The main characteristic of this immunologic disorder the simultaneous involvement of all the tissues of the nail unit.

Question 159

160. A- 4 year-old boy has a single circular alopecic atrophic patch on the vertex of the scalp. This most likely is due to which of the following conditions? A. Aplasia cutis congenita B. Congenital syphilis C. Keratosis Follicularis Spinulosa Decalvans D. Morphea en coup de sabre E. Triangular Alopecia

Answer 159

► A Aplasia cutis congenita often affects the vertex of the scalp and heals with a circular atrophic alopecic scar.

Question 160

161- A patient with curly hair that straightened after puberty, enamel hypoplasia, dental pits, and increased bone density likely has a mutation in which of the following genes? A. Distal-less homeobox-3 gene (DLX3) B. Vascular-endothelial growth factor receptor 3 C. Bone morphogenetic protein type 2 D. SLURP 1 E. SPINK 5

Answer 160

► A Distal-less homeobox-3 gene (DLX3) mutations cause trichodentoosseous syndrome, which is characterized by curly hair that straightens in the 2nd-3rd decades, enamel hypoplasia, dental pits, and increased bone density.

Question 161

162- A rapid onset of hair growth with or without accompanying virilization can occur in all of the following conditions EXCEPT: A. Adrenal adenoma B. Adrenal carcinoma C. Arrhenoblastoma D. Krukenburg tumor of the ovary E. Ovarian cyst

Answer 161

► E. Ovarian cyst All of these options except an ovarian cyst can cause a rapid onset of hair growth with or without accompanying virilization. An Arrhenoblastoma is a tumor of the ovary that secretes testosterone.

Question 162

163- Disorders associated with cortisol excess can cause hirsutism. All of the following clinical findings are associated with cortisol excess except: A. Plethora B. Centripetal obesity C. Striae D. Dorsocerical/supraclavicular fat pads E. Enlarged cystic ovaries

Answer 162

► E Plethora, centripetal obesity, striae and dorsocervical/supraclavicular fat pads are associated with signs of cortisol excess. Patients that have enlarged cystic ovaries are associated with polycystic ovarian disease.

Question 163

164- A 24 year-old over-weight woman presents with the complaint of hirsutism and asks about therapy. She has a virilization pattern of hair growth. On questioning, she relates having irregular menstrual periods and acne. Which of the following is the most likely diagnosis? A. Pheochromocytoma B. Polycystic ovarian disease C. Congenital adrenal hyperplasia D. Ovarian carcinoma E. Normal variant

Answer 163

► B This patients diagnosis is most reasonably Polycystic Ovarian disease and includes the features listed above in the question in addition to enlarged cystic ovaries, obesity and amenorrhea. Congenital adrenal hyperplasia can present with hirsutism and menstrual abnormalities, but usually not obesity. Ovarian carcinoma, pheochromocytoma and normal variant are not likely causes of this presentation.

Question 164

165- A 6-year old girl with atopic dermatitis presents with patchy non-scarring alopecia and nail pitting. You make the diagnosis of alopecia areata. What portion of the nail unit is responsible for her nail plate pitting: A. Hyponychium B. Proximal nail plate C. Proximal nail matrix D. Distal nail matrix E. Eponychium

Answer 164

► C The proximal nail matrix produce the dorsal nail plate. Defective keratinization in this region is therefore responsible for the pitting seen in alopecia areata, psoriasis, eczema, and other cutaneous diseases. The distal nail matrix produces the ventral nail plate and is visible as the lunula

Question 165

166- Transverse white bands on one or two nails is caused by: A. Trauma to the matrix B. Alopecia areata C. Psoriasis D. Systemic lupus erythematosis E. Lichen planus

Answer 165

► A Longitudinal leukonychia or transverse white bands are caused by trauma to the nail matrix. AA, psoriasis, SLE and LP all cause spotted red lunulae, not transverse white bands.

Question 166

167- Terry's nails are seen in which of the following conditions? A. Renal failure B. Pulmonary fibrosis C. Diabetes D. Bladder carcinoma E. Aerodigestive carcinoma

Answer 166

► C Terry's nails are described as all but the distal 2mm of each nail evenly white due to a defect in the nail bed. It can be seen in cirrhosis, congestive heart failure and diabetes.

Question 167

168- A patient with diffuse severe sudden hair loss developing 3 months after hospitalization for septicemia likely has which of the following conditions? A. Anagen effluvium B. Catagen effluvium C. Telogen effluvium D. Loose anagen syndrome E. Uncombable hair syndrome

Answer 167

► C Telogen effluvium typically ensues stressful events after a 2-3 month period.

Question 168

169- Triangular lunulae are: A. Part of the syndrome caused by a LMX1b mutation B. Associated with hyperplastic patellae C. Associated with a renal cell carcinoma D. Associated with a mutation of ATP2C1 E. Associated with a mutation of SERCA2

Answer 168

► A Triangular lunulae are part of the Nail-Patella syndrome. Findings in this syndrome include LMX1b mutation, hypoplastic or absent patellae, nephropathy, a "Lester Iris" - hyperpigmentation of the pupillary margin, cataracts, heterochromia irides and glaucoma. ATP2C1 is associated with Hailey hailey which encodes a golgi ATPase. ATP2A2 mutation causes Darier-White disease which encodes SERCA2 sarcoplasmic/endoplasmic reticulum calcium-pumping ATPase.

Question 169

170- Plummer's nails are associated with which of the following disorders? A. Dermatomyositis B. Systemic sclerosis C. Lupus erythematosus D. Hyperthyroidism E. Lead poisoning

Answer 169

► D Plummer's nails are onycholysis due to hyperthyroidism.

Question 170

171- Pathognomonic nails changes in HOOD syndrome or Hereditary osteoonychodysplasia are: A. Red and white longitudinal banding B. Pterygium inversum C. Yellow nails D. Pincer nails E. Triangular lunulae

Answer 170

► E In the HOOD syndrome (Hereditary osteoonychodysplasia) or the Nail-Patella syndrome, patients have different degrees of nail dysplasia. In general, the thumbs are most severely involved and nail disease decreases in severity from the second to the fifth digits. Characteristic nail changes include longitudinal ridging, koilonychia, and splitting. Toenails are rarely involved. Pathognomonic nail change is the V shaped triangular lunulae with the distal peak in the midline.

Question 171

172- A 44-year-old man undergoing chemotherapy for Hodgkin‟s lymphoma presents with horizontal rough bands involving the nail plates of all digits. What is the most likely disorder: A. Beau‟s lines B. Mee‟s lines C. Muehrcke‟s lines D. Onychophagia E. Dolichonychia

Answer 171

► A Beau‟s lines are transverse indented nail plate furrows caused by temporary growth arrest of the nail matrix, often due to chemotherapy or other stressful events and illnesses. Mee‟s lines are transverse white lines affecting all nails and growing out with the nail; there is no associated indentation. Mee‟s lines may be caused by arsenic poisoning, rheumatic fever, or other systemic diseases. Muehrcke‟s lines disappear with squeezing of the nail and are due to disorders of low albumin. Oncyhophagia refers to nail biting. Dolichonychia exhibits long, slender nails, and is associated with Ehlers-Danlos and Marfans.

Question 172

173- A patient that has Costello syndrome has thin anterior hair, redundant and thickened skin around neck. The patient has congenital myopathy, coarse facies, cardiac and developmental disability. The mutation is in: A. HRAS gene B. KRAS gene C. BRAF gene D. MEK1 gene E. MEK2 gene

Answer 172

► A Patient with Costello syndrome has a mutation in the HRAS gene. It is also known as faciocutaneoskeletal syndrome.

Question 173

174- Acquired progressive kinking of hair is a complication of which of the following medications? A. Antimetabolites B. Antimalarials C. Retinoids D. Sulfonamides E. Dapsone

Answer 173

► C Retinoids can cause acquired progressive kinking of hair.

Question 174

175- This term is used to describe the dorsal part of the digit that lies adjacent to the nail: A. Proximal nail fold B. Nail plate C. Nail matrix D. Nail bed E. Hyponychium

Answer 174

► A

Question 175

176- The combination of ankyloblepharon, ectodermal dysplasia, and cleft palate with wiry sparse hair is characteristic of which of the following syndromes? A. Focal Dermal Hypoplasia (Goltz syndrome) B. Tricho-rhino-phalangeal Syndrome C. Hay-Wells Syndrome D. Ectrodactyly-Ectodermal dysplasia-Clefting (EEC) E. Trichodentoosseous Syndrome

Answer 175

► C Hay-Wells Syndrome, also known as AEC (Ankyloblepharon-Ectodermal dysplasia-Clefting) is characterized by wiry, sparse hair or alopecia, ankyloblepharon, PPK, partial anhidrosis, cleft lip, palate, absent, and dystrophic nails.

Question 176

177- Frontal bossing, saddle nose, hypoplastic midface, peg shaped or conical teeth and hypopigmented short sparse scalp and body hair are prominent features of which of the following : A. Christ Siemens Touraine syndrome B. Hidrotic ectodermal dysplasia C. Arginosuccinic aciduria D. Monilethrix E. Pachyonychia congenita

Answer 176

► A Christ Siemens Touraine syndrome also known as Hypohidrotic ectodermal dysphasia or anhidrotic ectodermal dysplasia is X-linked recessive disorder that presents in infancy to early childhood. Features include hypo-anhidrosis with increased body temperature, hypopigmented sparse scalp and body hair, frontal bossing, saddle nose, hypoplastic midface, abnormal ears, hypo-anodontia, and increased bronchopulmonary inections. Cloustons syndrome or hidrotic ectodermal dysplasia is AD and associated with palmoplantar keratoderma, nail dystrophy, short sparse scalp hair, and tufting of the terminal phalanges. Arginosuccinic aciduria is associated with seizures, trichorrhexis nodosa, failure to thrive, and hyperammonemia. Monelothrix is associated with beaded hairs and keratosis pilaris. Pachyonychia congenita is associated with nail dystrophy and focal palmoplantar keratoderma.

Question 177

178. A- defect in which part of the nail cause a true leukonychia? A. Cuticle B. Hyponychium C. Lunula D. Nail bed E. Nail plate

Answer 177

► E True leukonychia is caused by an acute stress to the nail and manifests in the nail plate. Examples of true leukonychia are Mee's lines and punctate leukonychia. Apparent leukonychia, are a result of problems in the nail bed and are indicative of overall health compromise. Examples of apparent leukonychia include Lindsay's, Terrys and Muehrcke's nail.

Question 178

179-A patient is diagnosed with generalized congenital hypertrichosis lanuginosa has silvery, blond and gray hair over entire body. The acquired form is associated with: A. Fetal alcohol syndrome B. Ambras syndrome C. Cushings syndrome D. Congenital adrenal hyperplasia E. Arrhenoblastoma

Answer 178

► A Patients with generalized congenital hypertrichosis lanuginosa with silvery, blond, and gray hair over entire body. It is autosomal dominant with dental anomalies and the acquired form is associated with fetal hydantoin and fetal alcohol syndrome.

Question 179

180- All of the following are associated with anonychia or absence of the nail except: A. Nail-patella syndrome B. Congenital onychodysplasia of the index finger C. Coffin-Siris syndrome D. Onychodystrophy E. Proteus syndrome

Answer 179

► E All of the following except Proteus syndrome is associated with anonychia or absence of the nail.

Question 180

181- Tay’s syndrome is characterized by this hair condition : A. Trichorrhexis nodosa B. Trichorrhexis invaginata C. Trichoschisis D. Monilethrix E. Pili trianguli et canaliculi

Answer 180

► C Tay’s syndrome is also known as trichothiodystrophy which is characterized by trichoschisis or clean fracture of the hair shaft. I.e. brittle hair

Question 181

182- Anonychia is not a feature of which of the following syndromes? A. Hay Wells syndrome B. Coffin-Siris syndrome C. DOOR syndrome D. COIF syndrome E. Hidrotic ectodermal dysplasia

Answer 181

► E Hidrotic ectodermal dysplasia features hyperconvex nails, micronychia and nail dystrophy. All of the other listed options can cause anonychia. COIF syndrome is Congenital Onychodysplasia of the Index Finger and DOOR syndrome is Deafness, Congenital Onychodystrophy Recessive Form. Coffin-Siris syndrome is characterized by: nail onychodysplasia or aplasia (usually of the fifth finger or toe), coarse facies including bushy eyebrows, scant scalp hair, full lips, and microcephaly, mental/growth deficiency, short distal phalanges, and other abnormalities.Hay wells syndrome is ankyloblepharon ectyodermal defects cleft lip/palate syndrome and anonychia has been reported.

Question 182

183- Follicular atrophoderma is a feature of which of the following conditions? A. Bazex christol dupre syndrome B. Menkes kinky hair syndrome C. Papular atrichia D. Tricho-dento-osseous syndrome E. Tricho-rhino-phalangeal syndrome

Answer 182

► A Bazex christol dupre syndrome is characterized by follicular atrophoderma, hypotrichosis, hypohidrosis, and multiple basal cell carcinomas of face.

Question 183

184- This term is used to describe "angel wing" deformity with a gradual shortening of the proximal nail groove and leading to progressive thinning of the nail: A. Pterygium B. Beau's lines C. Onychorrhexis D. Brachyonychia E. Anonychia

Answer 183

► A Pterygium is angel wing deformity with gradual shortening of the proximal nail groove, leading to progressive thinning of the nail plate and secondary fissuring caused by the fusion of the proximal nail fold to the matrix and then to the nail bed.

Question 184

185- Ferritin levels need to be, at minimum, above which of the serum levels to treat iron-deficiency related telogen effluvium? A. 20ng/dL B. 40ng/dL C. 60ng/dL D. 30ng/dL E. 10ng/dL

Answer 184

► B The therapeutic target for iron deficiency related telogen effluvium is greater than 40mg/dL. Other causes of telogen effluvium include thyroid dysfunction, as well as such medications as beta- blockers, antihyperlipidemic drugs, and NSAIDs.

Question 185

A- child presents with sparse, fine hair, thin nails and loose skin in infancy. He has a pear- shaped, broad nose and on X-ray has cone-shaped epiphyses. Which of the following genes is mutated in this syndrome? A. P63 B. DLX3 C. RMRP D. TRPS1 E. ABCC6

Answer 185

► D TRPS1 is the gene mutated in Tricho-rhino-phalangeal syndrome. TRPS1 is a putative zinc finger transcription factor that is passed in an autosomal dominant fashion. P63 is mutated in Hay-Wells syndrome and Ectrodactyly-ectodermal dysplasia-clefting (EEC) dyndrome. DLX3 (distal-less homeobox-3) gene is associated with Trichodentoosseous syndrome. RMRP (RNA component of RNase MRP) is defective in Cartilage-Hair hypoplasia syndrome. ABCC6 is defective in pseudoxanthoma elasticum.

Question 186

187 -A subungual hematoma covering 50% of the nailbed should be managed by which of the following? A. No treatment B. Removal of the nail plate C. Trephination of the nail plate D. Needle aspiration of the hematoma E. Pressure dressing

Answer 186

► B Removal of the nail plate is necessary for management of hematomas covering more than 25% of the nailbed.

Question 187

188-Which autoantibody is associated with cuticular changes, shawl sign, and overall favorable prognosis? A. Mi-2 B. Jo-1 C. High titered ANA with speckled pattern D. Scl-70 E. Ro

Answer 187

► A Dermatomyositis has many different cutaneous manifestations including heliotrope rash, Gottron's papules and sign, mechanic's hand and poikiloderma atrophicans vasculare. Antibodies to Mi-2 in dermatomyositis are associated with cuticular changes, shawl sign and a overall favorable prognosis.

Question 188

179-A patient with a history of gastrointestinal polyposis presents with alopecia, generalized pigmentation, and nail dystrophy. Which of the following is the most likely diagnosis? A. Peutz-Jegher syndrome B. Gardner syndrome C. Cronkhite-Canada Syndrome D. Cowden syndrome E. Dyskeratosis congenita

Answer 188

►C Cronkhite-Canada Syndrome is a sporadic gastrointestinal polyposis syndrome characterized by alopecia, generalized pigmentation, and nail dystrophy.

Question 189

190- This term is used to describe a "tiger tail" or alternating bright and dark bands from a reduced sulfur content seen in Tay's syndrome A. Trichothiodystrophy B. Trichoschisis C. Pili torti D. Trichorrhexis nodosa E. Bamboo hair

Answer 189

► A A patient with Tay's syndrome can have trichothiodystrophy and looks like "tiger tail" with alterations of bright and dark bands. Other clinical findings in Tay's syndrome have ichthyosis, intellectual impairment, decreased fertility, and short stature.

Question 190

191- Regarding androgens in women, which of the following statements is NOT correct? A. Testosterone binds the androgen receptor B. Eyebrows, eyelashes and vellus hairs are androgen-dependent C. The hair follicle requires conversion of testosterone to dihydrotestosterone for expression of androgen action D. Dihydrotestosterone binds the androgen receptor E. There are no differences in eyelashes, eyebrows and vellus hair-bearing areas in men and women

Answer 190

► B Only testosterone and dihydrotestosterone bind the androgen receptor, Eyebrows, eyelashes and vellus hairs are NOT androgen-dependent, thus there are no differences between these areas in men and women, and the hair follicle requires conversion of testosterone to dihydrotestosterone for expression of androgen action.

Question 191

192. A woman with hypopigmented in lines of Blaschko and scarring alopecia likely suffers which of the following conditions? A. Chondrodysplasis punctata B. Anhidrotic Ectodermal Dysplasia C. Focal Dermal Hypoplasia D. Rothmund-Thomson Syndrome E. Bloch-Sulzberger Syndrome

Answer 191

► E Bloch-Sulzberger Syndrome (incontinentia pigmenti).

Question 192

193-A 24-year-old man with Lesch-Nyhan syndrome is most likely to present with what nail disorder: A. Beau‟s lines B. Mee‟s lines C. Muehrcke‟s lines D. Onychophagia E. Dolichonychia

Answer 192

► D Oncyhophagia refers to nail biting. Patient‟s with Lesch-Nyhan syndrome, caused by defective HGPRT enzyme, are prone to self-mutilating behaviors, including onychophagia. Beau‟s lines are transverse indented nail plate furrows caused by temporary growth arrest of the nail matrix, often due to chemotherapy or other stressful events and illnesses. Mee‟s lines are transverse white lines affecting all nails and growing out with the nail; there is no associated indentation. Mee‟s lines may be caused by arsenic poisoning, rheumatic fever, or other systemic diseases. Muehrcke‟s lines disappear with squeezing of the nail and are due to disorders of low albumin. Dolichonychia exhibits long, slender nails, and is associated with Ehlers-Danlos and Marfans.

Question 193

194- The epithelium that lies on the volar surface of the digit is the: A. Nail bed B. Nail matrix C. Nail plate D. Hyponychium E. Eponychium

Answer 193

► D The epithelium that lies on the most distal volar surface of the digit is the hyponychium. The nail plate is the actual nail made up of tightly packed onychocytes. The nail matrix is the epithelium which starts mid distal phalanx which generated the nail plate. It keratinizes without a granular layer and determines the thickness of the nail plate. The nail bed is the thin epithelium immediately beneath the nail plate.

Question 194

195- Patients that have a normal distal nail and a proximal nail that is white is associated with: A. Chronic renal failure B. Liver failure C. Pulmonary stenosis D. Gastrointestinal malignancy E. Tuberous sclerosis

Answer 194

► A Patients that have a normal distal nail and a proximal white nail have a condition called Lindsay's nails or half and half nail. The proximal nail that is white is from nail bed edema. This is from chronic renal failure.

Question 195

196- Air spaces in the hair shaft lead to this condition pictured below – name the condition: A. Pili trianguli et canaliculi B. Pili annulati C. Trichoschisis D. Pohl Pinkus constriction E. Wooly hair

Answer 195

► B Pili anulati is caused by air spaces in the hair shaft.

Question 196

197. A- patient is diagnosed with aplasia cutis congenita with a focal absence of epidermis on the scalp. This is also associated with: A. Adam Oliver syndrome B. Parry Romberg syndrome C. Cicatricial pemiphigoid D. Mastocytosis E. Epidermal nevi

Answer 196

► A Patients that are diagnosed with Adam Oliver syndrome have aplaisa cutis congenita over the posterior scalp and have terminal transverse defects of the limbs and small toenails. Most aplasia cutis congenita are on the scalp and most are single lesions. ACC can also be associated with Trisomy 13.

Question 197

198-How long should the last wash-out period before performing mycologic investigations in a clinically recurrent onychomycosis treated by terbinafine, itraconazole or fluconazole: A. One month B. Two months C. Three months D. Four months E. Six months

Answer 197

► C This is an interesting point, because we know that these systemic antifungals are retained in the keratin of the toenails up to six months after the treatment has been stopped. Therefore this answer could have been debatable were it not for the presence of fungi in the nail bed (which is the primary location of the disease) and where the scraping should be properly performed for mycologic testing after removal of a piece of nail as proximal as possible.

Question 198

199. A- 66-year-old woman presents with “black heads” on the nose. Examination reveals multiple small hairs protruding from the same follicle. What is the most likely associated diagnosis: A. Pili multigemini B. Pili bifurcati C. Trichostasis spinulosa D. Trichoschisis E. Trichothiodystrophy

Answer 198

► C Pili multigemini refers to a rare disorder of multiple divided hair matrices, with multiple hair shafts each with their own cuticle all arising from one follicle. Pili bifucarti exhibits hair shaft bifurcation into short segments along hair shafts, with each segment covered with its own cuticle. Trichostasis spinulosa refers to follicles filled with bundles of vellus hairs. This disorder may be associated with renal failure. Trichoschisis is a clean fracture of the hair shaft. Trichothiodystrophy is a disorder of beaded hair when viewed with polarizing light.

Question 199

200-The most common cultured agent in chronic paronychia associated with a black hue of the lateral edge of the nail plate is: A. Trichophyton rubrum B. gram-negative bacteria C. Candida spp D. Scytalidium dimidiatum E. Scopulariopsis brevicaulis

Answer 199

► D Chronic paronychia is likely a condition that represents a contact reaction to allergens or irritants. It often is superinfected. Scytalidium dimidiatum causes a black discolored nail

Question 200

201. A- patient with diffuse hair loss developing after a thallium scan likely has which of the following conditions? A. Anagen effluvium B. Catagen effluvium C. Telogen effluvium D. Loose anagen syndrome E. Uncombable hair syndrome

Answer 200

► A Anagen effluvium can result from infusions of thallium.

Question 201

202- The combination of a low-set hairline and synophrys is seen in which of the following conditions? A. Werner syndrome B. Turner Syndrome C. Noonan Syndrome D. Cornelia de Lange Syndrome E. Waardenburg's syndrome

Answer 201

► D Cornelia de Lange Syndrome is a sporadic disorder that may be caused by mutations in nippedBeta- like gene. Clinically, patients have cutis marmorata, hirsutism, synophrys, mental retardation, short stature, GU abnormalities, hearing loss, and congenital heart defects.

Question 202

203- The common culprit of proximal white subungual onychomycosis is : A. Candida albicans B. Trichophyton rubrum C. Trichophyton interdigitale D. Scydalidium dimidiatum E. Fusarium spp

Answer 202

► B T. rubrum is responsible for more than 95% of these cases.

Question 203

204- This term is to describe hair loss mostly on the vertex of the scalp in black females associated with chemical relaxers: A. Follicular degeneration syndrome B. Pseudopelade of Broq C. Folliculitis decalvans D. Dissecting folliculitis E. Traction alopecia

Answer 203

► A Hair loss on the vertex of the scalp in black females also known as hot comb alopecia is follicular degeneration syndrome. It can occur with chemical relaxers and premature desquamation of the inner root sheath with fibrosis.

Question 204

205- This is a small reddish-blue tender subungual tumor that can affect the nails: A. Glomus tumor B. Myxoid cyst C. Acquired digital fibrokeratoma D. Accessory digit

Answer 204

► A A glomus tumor is a reddish subungual tumor that can affect the nail. On pathology, one can see small uniform round cells with eosinophilic cytoplasm.

Question 205

206. A- 36-year-old woman presents with hair loss. Examination of a single follicle reveals multiple hair shafts protruding, each surrounded by its own cuticle. What is the most likely diagnosis: A. Pili multigemini B. Pili bifurcati C. Trichoptilosis D. Trichoschisis E. Trichothiodystrophy

Answer 205

► A Pili multigemini refers to a rare disorder of multiple divided hair matrices, with multiple hair shafts each with their own cuticle all arising from one follicle. Pili bifucarti exhibits hair shaft bifurcation into short segments along hair shafts, with each segment covered with its own cuticle. Trichoptilosis refers to “split ends” of the distal portion of the hair shaft. Trichoschisis is a clean fracture of the hair shaft. Trichothiodystrophy is a disorder of beaded hair when viewed with polarizing light.

Question 206

207-Blue lunulae are associated with each of the following except: A. Argyria B. Cardiac failure C. Quinacrine D. Wilson‟s disease E. Phenolphthalein

Answer 206

► B Blue lunulae have been reported in association with argyria, Wilson‟s disease, hereditary acrolabial telangectasia, paronychia, phenolphthalein, quinacrine, topical bichloride, and mercury exposure.

Question 207

207- The best test for Cushing syndrome is: A. Plasma LH B. Plasma testosterone C. Plasma prolactin D. Plasma DHEA E. Overnight dexamethasone suppresion test

Answer 207

► E The overnight dexamethasone suppression test is the test of choice for diagnosis of Cushing syndrome. In this test, a dose of dexamethasone is given at 11pm and the plasma cortisol level is drawn the following morning. If the Cushing syndrome is from an adrenal tumor or an ectopic ACTH producing tumor, there is no change in cortisol secretion. A normal response is suppression of the cortisol level by the extra dexamethasone. If the Cushings is from a pituitary tumor, at low doses of dexamethasone there are no changes in cortisol levels while at high doses of dexamethasone there will be a normal suppression. The other listed items are not used for diagnosis of Cushing syndrome.

Question 208

209- Steatocystoma multiplex and natal teeth are associated with which of the following conditions? A. Jadassohn-Lewandowsky pachyonychia congenita (Type 1) B. Jackson-Lawler pachyonychia congenita (Type 2) C. Nail-patella syndrome D. Coffin-Siris syndrome E. Rubenstein-Taybi syndrome

Answer 208

►B Jackson-Lawler pachyonychia congenita (Type 2) is characterized by thickening of the nailbed and plate, steatocystoma multiplex, and natal teeth.

Question 209

210- Which of the following is NOT a cause of red lunulae? A. Endocarditis B. Psoriasis C. Carbon monoxide poisoning D. Imuran E. Rheumatoid arthritis

Answer 209

► A Endocarditis is not a cause of red lunulae. Alopecia areata, psoriasis, prednisone for connective tissue diseases, carbon monoxide poisoning, cardiac failure, SLE, RA, COPD, CO2, cirrhosis, imuran and radiation are the causes of this condition.

Question 210

211- What is the mode of inheritance of(Monilethrix) a disorder of the hair shaft? A. Autosomal dominant B. Autosomal recessive C. X-linked dominant D. X-linked recessive E. Sporadic

Answer 210

► A Monilethrix is an autosomal dominant condition with variable penetrance. The hair shaft has an elliptical or beaded hair appearance.

Question 211

212- Highest graft survival in hair transplantation is achieved through the use of: A. 4-5 mm plugs B. Minigrafts C. Single hairs D. Follicular units E. Micrografts

Answer 211

► D Seager et. al examined the survival rate of single hair grafts and compared them to follicular unit grafts in a single patient. They found that there was a much higher survival rate in the follicular unit grafts. They hypothesized that the extra tissue surrounding the follicular unit grafts protected them from crush injury

Question 212

213- This describes a patch of vellus hairs or complete hair loss in the frontal-temporal region with no treatment available and usually persistent. This condition is called: A. Triangular alopecia B. Loose anagen syndrome C. Telogen effluvium D. Anagen effluvium E. Trichotillomania

Answer 212

► A Triangular alopecia is also known as temporal alopecia and describes a triangular patch of vellus hairs or complete hair loss in the frontal-temporal region. On histology there is vellus hairs seen and there is usually no treatment and is persistent.

Question 213

214- This is to describe a mutation in the hairless gene and is characterized by universal alopecia right after birth. Patients with this condition have normal eyelash, cornified cysts over face and neck. This describes: A. Papular atrichia B. Acquired progressive kinking C. Trichotillomania D. Woolly hair nevus E. Ichthyosis follicularis, atrichia, photophobia

Answer 213

► A Patients with papular atrichia have an autosomal recessive mutation in the hairless gene. They have universal alopecia right after birth. They have normal eyelash, cornified cysts over face and neck.

Question 214

215- The most common enzyme abnormality in congenital adrenal hyperplasia is: A. 3-beta-hydroxysteroid dehydrogenase isomerase B. 11-beta-hydroxylase C. 21-hydroxylase D. 15-hydroxylase E. 17-hydroxylase

Answer 214

► C 21- hydroxylase deficiency is present in 95% of cases of congenital adrenal hyperplasia. This defect in adrenal steroidogenesis can occur at any point in life, but affected girls will generally present around puberty with hirsutism and mentsrual irregularity/primary amenorrhea. The key feature is excess androgen production.

Question 215

216- Atrophy of the isthmus is a characteristic histologic finding of which of the following conditions? A. Discoid lupus erythematosus B. Lichen planopilaris C. Parry-Romberg syndrome D. Folliculitis Decalvans E. Acne Keloidalis Nuchae

Answer 215

► B Lichen planopilaris is characterized by perifollicular lymphocytic infiltration with atrophy of the isthmus.

Question 216

217- Triangular lunulae are seen in what disorder? A. Dyskeratosis congentia B. Neurofibromatosis II C. Gorlin”s syndrome D. Nail-patella syndrome E. Papillon-Lefevre syndrome

Answer 216

► D Triangular lunulae are seen in nail-patella syndrome which is characterized by mutation in LMX1b.

Question 217

218- Spotted red lunulae are absent in which of the following conditions? A. Alopecia areata B. Keratosis follicularis C. Systemic lupus erythematosus D. Rheumatoid arthritis E. Lichen planus

Answer 217

► B Iron deficiency can lead to spoon nail deformity or koilonychias

Question 218

219- Distal subungual onycholysis associated with paronychia is due to: A. Candida spp B. Fusarium spp C. Scytalidium dimidiatum D. Trichophyton rubrum E. Trichophyton mentagrophytes

Answer 218

► C This mold is the only one responsible for paronychia in DLSO and is more often observed in finger than in toenails.

Question 219

220- A 14-year-old man with a history of extensive striae, upward lens dislocation, and pectus excavatum would be most likely to present with what nail abnormality: A. Beau‟s lines B. Mee‟s lines C. Muehrcke‟s lines D. Onychophagia E. Dolichonychia

Answer 219

► E Beau‟s lines are transverse indented nail plate furrows caused by temporary growth arrest of the nail matrix, often due to chemotherapy or other stressful events and illnesses. Mee‟s lines are transverse white lines affecting all nails and growing out with the nail; there is no associated indentation. Mee‟s lines may be caused by arsenic poisoning, rheumatic fever, or other systemic diseases. Muehrcke‟s lines disappear with squeezing of the nail and are due to disorders of low albumin. Oncyhophagia refers to nail biting. Dolichonychia exhibits long, slender nails, and is associated with Ehlers-Danlos and Marfans.

Question 220

221- Mutations in c-kit are associated with which of the following conditions? A. Waardenburg syndrome B. Piebaldism C. Tuberous sclerosis D. Vogt-koyanagi-harada E. Cornelia de Lange Syndrome

Answer 220

► B Piebaldism is caused by mutations in c-kit.

Question 221

222- Splinter hemorrhage of the nail can be seen with which of the following parasitic infections? A. Scabies B. Trichinosis C. Sparganosis D. Dracunculiasis E. Gnathostomiasis

Answer 221

► B Trichinosis can cause splinter hemorrhage of the nails.

Question 222

223- Which of the following is caused by mutations in gap junction proteins? A. Rothmund-Thompson syndrome B. Hidrotic ectodermal dysplasia C. Anhidrotic ectodermal dysplasia D. Netherton syndrome E. Naxos syndrome

Answer 222

► B Hidrotic ectodermal dysplasia is caused by mutations in connexin 30, which is a gap junction protein.

Question 223

224- Which palmoplantar keratoderma is characterized by painful hyperkeratosis? A. Richner-Hanhart B. Howell-Evans C. Vohwinkel D. Unna-thost E. Mal de Meleda

Answer 223

► A While most palmoplantar keratodermas are characterized by thick plaques of the palms and soles that are usually painless, Richner Hanhart is classically associated with painful lesions, rendering it distinctive. Richner Hanhart is also associated with a painful keratitis and pigment dilution of the hair.

Question 224

225- Proximal white subungual onychomycosis with polydactylous involvement needs at least one laboratory test: A. Blood count B. Sedimentation rate C. T-helper CD4 lymphocytes D. Liver enzymes E. HIV

Answer 224

► E This type of onychomycosis has a prognostic value: It means that the CD4 lymphocytes are below 450.

Question 225

226- Fungal finger onycholysis usually results from: A. Trichophyton rubrum B. Trichophyton interdigitale C. Candida albicans D. Scopulariopsis brevicaulis E. Fusarium spp

Answer 225

► C It is widely accepted that C. albicans acts as colonizer that has found an ideal environment.

Question 226

227- Thin hair with premature graying is characteristic of which of the following syndromes? A. Werner syndrome B. Turner Syndrome C. Noonan Syndrome D. Cornelia de Lange Syndrome E. Waardenburg's syndrome

Answer 226

► A Werner syndrome is characterized by premature degeneration of major organs that loosely mimics premature aging. It is associated with sclerodermoid changes, leg ulcerations, premature graying, cataracts, premature atherosclerosis,a dn diabetes. It is associated with defects in the helicase RecQL2.

Question 227

228- A patient with a history of thin, short, and easily fractured hairs comes to your clinic. Trichoscopy reveals the following findings. What is the most likely diagnosis? A. Trichorrhexis nodosa B. Monilethrix C. Pili torti D. Trichoclasis E. Pili annulati

Answer 227

Correct choice: B. Monilethrix Explanation: Monilethrix is a rare cause of hair thinning and characterized by beading or a “regularly bended ribbon” appearance under trichoscopy. Trichorrhexis nodosa is characterized by frayed ends resembling "broom-sticks". Pili torti is characterized by hair twisted at intervals of 90 to 360 degrees. Trichoclasis is also known as split ends. Pili annulati is characterized by alternating light and dark bands secondary to air spaces.

Question 228

229- You see this finding on a new patient. What is the most likely gene mutation? A. ATP2C1 B. ATP2A2 C. SERCA2 D. TSC1 E. TSC2

Answer 228

Correct choice: B. ATP2A2 Explanation: V-shaped nicking is suggestive of Darier’s disease. This is due to a mutation in ATP2A2, which encodes the protein, SERCA2, for transport of calcium from the cytosol into the endoplasmic reticulum of the cell. ATP2C1 mutation occurs in Hailey Hailey disease. SERCA2 is the protein mutated in Darier's disease. TSC1 and TSC2 are mutated in Tuberous Sclerosis.

Question 229

230- An 18 year-old girl who was hospitalized last month after a serious car accident is noted to have white transverse grooves on her fingernails and toenails. The most likely diagnosis is: A. Twenty-nail dystrophy B. Beau's lines C. Half-and-half nails D. Mees' lines E. Terry's nails

Answer 229

Correct choice: B. Beau's lines Explanation: Beau's lines are transverse grooves in the nails. They are caused by a generalized systemic condition which disrupts nail formation. Conditions may include infection, myocardial infection, neurologic events and cytotoxic medications.

Question 230

231- The best diagnosis for this scarring form of alopecia is: A. Chronic cutaneous lupus erythrematosus B. Lichen planopilaris C. Pseudopelade of Broq D. Perifolliculitis capitis abscedens et suffodiens E. Central centrifugal cicatricial alopecia

Answer 230

Correct choice: D. Perifolliculitis capitis abscedens et suffodiens Explanation: Perifolliculitis capitis abscedens et suffodiens is also known as dissecting cellulitis of scalp and is uncommon chronic suppurative disorder that most often affects young black men. Together with acne conglobata, hidradenitis suppurativa and pilonidal cysts, it is a component of the 'follicular occlusion tetrad" in dermatology. Patients present with multiple painful inflammatory nodules and fluctuant abscesses of the scalp, which are often interconnected via sinus tracts. The vertex and occiput are the sites of predilection. As the disease progresses, scarring alopecia may develop.

Question 231

232- A 24 year-old overweight woman presents with the complaint of hirsutism and asks about therapy. She has a virilization pattern of hair growth. On questioning, she notes having irregular menstrual periods and acne. Which of the following is the most likely diagnosis? A. Pheochromocytoma B. Polycystic ovary syndrome C. Congenital adrenal hyperplasia D. Ovarian carcinoma E. Normal variant

Answer 231

Correct choice: B. Polycystic ovary syndrome Explanation: This patients diagnosis is most likely Polycystic Ovary Syndrome (PCOS) and includes the features listed above in the question in addition to enlarged cystic ovaries and potentially amenorrhea. Congenital adrenal hyperplasia can present with hirsutism and menstrual abnormalities, but usually not obesity. Ovarian carcinoma, pheochromocytoma and normal variant are not likely causes of this presentation.

Question 232

233- A 66-year-old woman presents with “black heads” on the nose. Examination with magnification reveals multiple small hairs protruding from the same follicle. What is the most likely associated diagnosis? A. Pili multigemini B. Pili bifurcati C. Trichostasis spinulosa D. Trichoschisis E. Trichothiodystroph

Answer 232

Correct choice: C. Trichostasis spinulosa Explanation: Trichostasis spinulosa refers to follicles filled with bundles of vellus hairs, these appear to be black heads but on closer inspection short fine hairs protrude from the follicle. This disorder may be associated with renal failure. Pili multigemini refers to a rare disorder of multiple divided hair matrices, with multiple hair shafts each with their own cuticle all arising from one follicle--You actually see the dark terminal hairs coming out. Pili bifucarti exhibits hair shaft bifurcation into short segments along hair shafts, with each segment covered with it’s own cuticle. Trichoschisis is a clean fracture of the hair shaft. Trichothiodystrophy is a disorder of beaded hair when viewed with polarizing light.

Question 233

234- Which of the following choices is FALSE regarding this diagnosis? A. This condition most commonly occurs on the thumbnails. B. This may be associated with trauma. C. Isotretinoin has been shown to be an effective treatment for this. D. Evaluation of the nail fold helps to distinguish this diagnosis from others in the differential. E. This condition is often symmetric.

Answer 233

Correct choice: C. Isotretinoin has been shown to be an effective treatment for this. Explanation: The correct answer is C (C is false). This photo represents median nail dystrophy, which is most common on the thumbs and is often symmetric. It may be associated with isotretinoin use or antecedent trauma. The main differential diagnosis is habit tic deformity, which often shows an enlarged lunula, loss of the cuticle, and proximal nail fold changes (traumatic). This other options listed are features seen in median nail dystrophy.

Question 234

235- A 36-year-old woman presents with arthralgias and three fingernails exhibiting adherence of the ventral surface of the distal nail plate to the hyponychium. What is the most likely associated disease: A. Psoriasis B. Lichen Planus C. Systemic Sclerosis D. Yellow Nail Syndrome E. Multicentric Reticulohistiocytosis

Answer 234

Correct choice: C. Systemic Sclerosis Explanation: The most common cause of ventral pterygium is systemic sclerosis. Lichen planus is most commonly associated with dorsal, rather than ventral, pterygium. Multicentric reticulohistiocytosis is associated with deforming arthritis and “coral bead” nodules around the proximal nail fold, but not with pterygium.

Question 235

236- Biopsy reveals absence of follicles, a mixed inflammatory infiltrate, and dermal fibrosis. Which of the following is the most likely diagnosis? A. Folliculitis decalvans B. Cicatricial pemphigoid C. Pustular psoriasis D. Dissecting cellulitis E. Erosive pustular dermatosis

Answer 235

Correct choice: E. Erosive pustular dermatosis Explanation: Erosive pustular dermatosis of the scalp (EPDS) is a rare, inflammatory, and chronic slow-onset disease that causes scarring alopecia and mainly affects the scalp of older men. Clinical diagnosis can be challenging since it mimics more common diseases and due to its nonspecific clinical features. Different histologic findings can be seen depending on the stage of disease, with EPDS potentially being characterized by an atrophic epidermis and chronic inflammation consisting of lymphocytes, neutrophils, and occasional foreign body giant cells. Dermoscopy (or trichoscopy) has been recently reported as a useful aid for the diagnosis, as it shows absent follicular ostia with marked skin atrophy, with consequent visualization of dermal vessels, although histology is mandatory for the final diagnosis. The remaining answer choices are reasonable to include on the differential diagnosis, but the clinico-pathologic findings are most supportive of EPDS.

Question 236

. Patient presents with longstanding melanonychia. Where should you biopsy? A. Nail matrix B. Nail plate C. Nail bed D. Proximal nail fold E. Lateral nail fold

Answer 236

Correct choice: A. Nail matrix Explanation: Melanocytic processes are found in the nail matrix, where melanocytes reside.