Disorders of thhe Hair and Nails Flashcards
- 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
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.
- 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
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.
- Blue lunulae may result from which of the following?
A. Chronic bronchiectasis
B. Hydroxyurea
C. Nail-patella syndrome
D. Renal failure
E. Alopecia areata
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.
- 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
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.
- 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
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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.
- Splinter hemorrhage of the nail can be seen with which parasitic infections?
A. Scabies
B. Trichinosis
C. Sparganosis
D. Dracunculiasis
E. Gnathostomiasis
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.
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- 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
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.
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- 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
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.
- Which of the following nutritional deficiencies is associated with perifollicular hemorrhage?
A. Vitamin C
B. Niacin
C. Vitamin B12
D. Folate
E. Zinc
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.
- 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
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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.
- 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
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.
- 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
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.
- 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
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.
- 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
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.
- 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
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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.
- 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
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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.
- 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
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.
- 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
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.
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- 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
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.
- 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
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
- 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
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.
- 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
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.
- 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
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.
- 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
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.
- 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
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.
- 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
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.
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- 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
Correct choice: D. Deafness
Explanation: Deafness and pili torti are the two cardinal features of Björnstad syndrome.
- 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
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
- Muehrcke’s nails are most often associated with:
A. Hyperthyroidism
B. Hypothyroidism
C. Hypoalbuminemia
D. Hyperalbuminemia
E. Chronic renal failure
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.
- The visible portion of the nail matrix is called the:
A. Eponychium
B. Lunula
C. Cuticle
D. Hyponychium
E. Nail plate
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.
- 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
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.
- 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
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.
- 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
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.
- What is the most likely diagnosis?
A. Bjornstad syndrome
B. Trichothiodystrophy
C. Uncombable hair syndrome
D. Monilethrix
E. Netherton’s syndrome
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
- 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
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.
- 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
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.
- 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
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.
- 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
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.
- 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
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.
- 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
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.
- Black nail is caused by:
A. Trichophyton mentagrophytes
B. Proteus mirabilis
C. Pseudomonas spp.
D. Staphylococcus aureus
E. Hortaea werneckii
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.
- 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
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.
- 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
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.
- 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
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.
- 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
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.
- 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
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
- Fraying of the cuticles occurs in which condition?
A. Dermatomyositis
B. Systemic sclerosis
C. Lupus erythematosus
D. Hypothyroidism
E. Lead poisoning
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.
- 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
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.
- 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
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.
- 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
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.
- 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
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.
- Menkes kinky hair syndrome is associated most commonly with:
A. Trichorrhexsis invaginata
B. Trichostasis spinulosa
C. Pili multigemini
D. Pili torti
E. Plica neuropathica
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.
- 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)
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.
- 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
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.
- 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
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.
- 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
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.
- 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
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.
- 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
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.
- 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
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.
- 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
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
- 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
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.
- Endonyx onychomycosis is most commonly due to:
A. Trichophyton rubrum
B. Trichophyton tonsurans
C. Trichophyton soudanense
D. Scytalidium dimidiatum
E. Scopuliaropsis
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.
- 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
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.
- 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
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.
- 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
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.
- 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
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.
- 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
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.
- 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
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.
- Which of the following are findings associated with Nail-Patella Syndrome?
A. Glomerulonephritis
B. Alopecia
C. Bladder diverticuli
D. Photosensitivity
E. Hip dysplasia
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.
- 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
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.
- 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
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
- 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
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.
- 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
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).
- 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
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.
- 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
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.
Which of the following is not associated with increased fragility?
A. Trichorrhexis invaginata
B. Monilethrix
C. Pili torti
D. Trichorrhexis nodosa
E. Pili multigemini
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.
- 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
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.
- The diagnosis is
A. Psoriasis
B. Alopecia areata
C. Onychomycosis
D. Lichen planus
E. Tetracycline-induced photo-onycholysis
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.
- 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
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.
- 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
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.
- 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
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.
- 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
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.
- 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
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.
- 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
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.
- 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
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.
- 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
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.
- 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
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.
- 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
Correct choice: E. Lymphedema and bronchiectasis
Explanation: Lymphedema and bronchiectasis are associated with yellow nail syndrome.
- This hair finding is caused by mutations in what gene?
A. SPINK 5
B. NEMO
C. SERCA2
D. Dyskerin
E. Keratin 16
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.
- 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
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.
- 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
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.
- 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
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).
- 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
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.
- Trichobezoar is a potentially life-threatening complication of:
A. Alopecia areata
B. Anhidrotic ectodermal dysplasia
C. Trichotillomania
D. Discoid lupus
E. Lichen planopilaris
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.
- 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
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.
- 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
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.
- 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
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.
- What nutritional deficiency can lead to this nail finding?
A. Vitamin B12
B. Vitamin C
C. Iron
D. Magnesium
E. Thiamine
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.
- 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
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.
- 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
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.
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
► 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.
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
► B
Pili anulati is caused by air spaces in the hair shaft.
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
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.
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
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.
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
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.
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.
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.
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
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.
. 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
Correct choice: A. Nail matrix
Explanation: Melanocytic processes are found in the nail matrix, where melanocytes reside.
