Musculo - Pathology (Part 2) Flashcards
Hypercalcemia associated with sarcoidosis occurs because of the increased conversion of what substance into its active form in epithelioid macrophages?
Vitamin D
What type of arthritis is associated with sarcoidosis?
Rheumatoid arthritis (remember: GRAIN (Gammaglobulinemia, Rheumatoid arthritis, ACE increase, Interstitial fibrosis, Noncaseating granulomas)
What is the treatment for sarcoidosis?
Steroids
Polymyositis involves progressive _____ (proximal/distal) muscle weakness.
Proximal
Which part of the immune system causes damage in polymyositis?
CD8+ T-cell-induced injury to myofibers
Polymyositis most often involves what body part?
Shoulders
Dermatomyositis is similar to polymyositis but also involves what skin symptom?
Rash (heliotrope, malar and shawl and face)
Which lab results are abnormal in polymyositis/dermatomyositis?
Elevated creatine kinase, elevated aldolase, positive antinuclear antibody, and anti-Jo-1
What is the diagnostic procedure for dermatomyositis and polymyositis?
Muscle biopsy
What is the treatment for polymyositis and dermatomyositis?
Steroids are used to treat both disorders
Gottrons papules are symptoms of what disease?
Dermatomyositis
While the symptoms of myasthenia gravis _____ (improve/worsen) with muscle use, symptoms of Lambert-Eaton syndrome _____ (improve/worsen) with muscle use.
Worsen; improve
Myasthenia gravis is caused by autoantibodies to which receptor?
Acetylcholine receptors
Name two ophthalmic manifestations of myasthenia gravis.
Ptosis and diplopia
Myasthenia gravis is associated with which type of tumor?
Thymoma
Individuals with myasthenia gravis complain of generalized weakness that is worse in the _____ (morning/evening).
In myasthenia gravis, weakness tends to be worse toward the end of the day
Lambert-Eaton syndrome is caused by autoantibodies to which types of ionic channels, leading to a decrease in acetylcholine release?
Calcium channels
Lambert-Eaton syndrome leads to _____ (proximal/distal) muscle weakness.
Proximal
Which malignancy is most closely associated with Lambert-Eaton syndrome?
Small-cell lung cancer
True or False? Both myasthenia gravis and Lambert-Eaton syndrome improve with acetylcholinesterase inhibitor use.
False; although myasthenia gravis symptoms reverse with the use of acetylcholinesterase inhibitors, there is no reversal in Lambert-Eaton syndrome with acetylcholinesterase inhibitors alone such as edrophonium
While myasthenia gravis involves autoantibodies against the _____ (presynaptic/postsynaptic) acetylcholine receptors, Lambert-Eaton syndrome involves autoantibodies against the _____ (presynaptic/postsynaptic) calcium channels.
Postsynaptic; presynaptic
In contrast to myasthenia gravis, Lambert-Eaton syndrome spares what muscle group?
Extraocular muscles
What five characteristics are associated with mixed connective tissues diseases?
Raynauds phenomenon, Fatigue, Arthralgias, Myalgias, and Esophageal hypomotility (remember: Raynauds FAME)
Patients with mixed connective tissue disease are positive for _____ autoantibodies.
U1 ribonucleoprotein
What is the treatment for mixed connective tissue disease?
Steroids
What condition involves excessive fibrosis and collagen deposition throughout the body but most commonly under the skin?
Scleroderma
Does scleroderma affect more men or women?
Women
Although scleroderma most commonly affects the skin, what four other organ systems can it affect?
Cardiovascular, pulmonary, gastrointestinal, and renal
Describe the progression of diffuse scleroderma.
Widespread skin involvement, rapid progression, and early visceral involvement
What are the two types of scleroderma?
Diffuse scleroderma and CREST syndrome
Diffuse scleroderma is associated with which antibody?
Anti-Scl-70 antibody (anti-DNA topoisomerase I antibody)
What does CREST stand for?
Calcinosis, Raynauds phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasia
A patient presents for examination. Although middle-aged, she has no wrinkles (denies plastic surgery) and her skin is puffy and taut. She also complains of difficulty swallowing. From what disease is she suffering?
Scleroderma
Skin involvement in CREST syndrome is often confined to which two areas of the body?
Fingers and face
Which has a more benign course: diffuse scleroderma or CREST syndrome?
CREST syndrome
Which antibody is associated with CREST syndrome?
Anticentromere antibody (remember: C for CREST)
What is a lipoma?
A soft, well-encapsulated tumor derived from fat
True or False? Lipoma recurrences are common even after excision.
False; simple excision of lipomas is usually curative
While lipomas are _____ (benign/malignant) in nature, liposarcomas are _____ (benign/malignant).
Benign; malignant
Rhabdomyoma is a benign tumor derived from what kind of muscle?
Striated (skeletal or cardiac) muscle
Rhabdomyoma of the heart is associated with which genetic disease?
Tuberous sclerosis
What is the most common malignant soft tissue tumor of childhood?
Rhabdomyosarcoma
Rhabdomyosarcomas often arise from skeletal muscle from what two locations in the body?
Head and neck
_____ (Macules/Patches) are flat discolorations of the skin <1 cm in size, while _____ (macules/patches) represent a similar lesion except they are > 1 cm in size.
Macules; patches
_____ (Papules/Plaques) are elevated skin lesions < 1 cm in size, while _____ (papules/plaques) represent a similar lesion but are > 1 cm in size.
Papules; plaques
_____ (Bullae/Vesicles) are small fluid-containing blisters, while _____ (bullae/vesicles) are large fluid-containing blisters.
Vesicles; bullae
Which dermatologic term describes a transient vesicle?
Wheal
What irregular, raised lesion results from scar tissue hypertrophy following trauma to skin, especially in African-Americans?
Keloid
What is the medical term for a blister containing pus?
Pustule
Dried exudate from a vesicle, bulla, or pustule is called a _____.
Crust
An increased thickness of the stratum corneum is known as _____.
Hyperkeratosis
Which phenomenon describes hyperkeratosis with retention of nuclei in stratum corneum, as seen in psoriasis?
Parakeratosis
While acantholysis is the _____ (hyperplasia/separation) of epidermal cells, acanthosis represents epidermal _____ (hyperplasia/separation).
Separation; hyperplasia
Tinea versicolor is an example of what type of dermatologic lesion?
Macule
Acne vulgaris is an example of what type of dermatologic lesion?
Papule
Psoriasis causes what gross and microscopic dermatologic lesions?
Plaques, parakeratosis and hyperkeratosis
Chickenpox is an example of what type of dermatologic lesion?
Vesicle
Hives are an example of what type of dermatologic lesion?
Wheal
Pemphigus vulgaris causes which pathologic change of the epidermis?
Acantholysis
Another name for warts, _____ are soft, tan-colored, cauliflower-like lesions of the skin.
Verrucae
Name three histopathologic findings in verrucae.
Epidermal hyperplasia, hyperkeratosis, and koilocytosis
Warts found on the hands are referred to as _____ _____, while warts found on the genitals are called _____ _____.
Verruca vulgaris; condyloma acuminatum
Condyloma acuminatum, or genital warts, are caused by what virus?
Human papillomavirus
What is the lay term for a nevocellular nevus, a benign lesion?
Mole
Hives, also known as _____, are pruritic wheals that form secondary to degranulation of what type of cell?
Urticaria; mast cells
A freckle, a lesion that results from increased melanin pigment, is also known as _____.
Ephelis
In the case of atopic dermatitis (eczema), pruritic eruption takes place on which surfaces of the body?
Flexor surfaces
Atopic dermatitis is often associated with which two other atopic diseases?
Asthma and allergic rhinitis
Allergic contact dermatitis is a type _____ hypersensitivity reaction that follows exposure to an allergen.
IV
Acanthosis with parakeratotic scaling that is particularly found on knees and elbows is called what?
Psoriasis
An increased stratum spinosum and a decreased stratum granulosum is seen with which skin disorder?
Psoriasis
A woman develops plaques and papules with silvery scaling on her elbows and knees. Pinpoint bleeding occurs when a scale is removed. What is the name of the sign describing the latter phenomenon? What is the most likely diagnosis?
Auspitz sign; psoriasis
Which skin disorder is described as flat, pigmented, squamous epithelial proliferation with keratin-filled cysts?
Seborrheic keratosis
What is the Sign of Leser-Trélat?
The sudden appearance of multiple seborrheic keratoses indicating underlying malignancy
In albinism, melanocyte number is _____ (increased/decreased/unchanged), while in vitiligo melanocyte number is _____ (increased/decreased/unchanged).
Unchanged (ie normal); decreased
In albinism, decreased melanin production is due to what?
Decreased activity of tyrosinase
What developmental failure can result in albinism?
Failure of neural crest cell migration during development
How does vitiligo appear clinically?
As irregular areas of complete skin depigmentation
Melasma, or skin hyperpigmentation, is associated with what two situations?
Pregnancy and oral contraceptive use
Name two organisms that commonly cause impetigo.
Staphylococcus aureus and Streptococcus pyogenes
Which infectious superficial skin infection is commonly characterized by honey-colored crusting?
Impetigo
Cellulitis, an acute painful spreading infection of the dermis and subcutaneous tissues, is most commonly caused by which two organisms?
Staphylococcus aureus and Streptococcus pyogenes
Name the deep tissue infection caused by flesh-eating bacteria that results in crepitus due to bacterial production of carbon dioxide and methane.
Necrotizing fasciitis
Which bacteria cause necrotizing fasciitis?
Anaerobic bacteria and Streptococcus pyogenes
Staphylococcal scalded skin syndrome is caused by a bacterial product called an _____ that destroys the keratinocyte attachments in the stratum _____ only.
Exotoxin; granulosum
A parents calls the pediatrician because her newborn has a fever and rash. Every time she picks the baby up, it seems like her skin peels off. From what is the baby suffering?
Staphylococcal scalded skin syndrome
Which virus causes hairy leukoplakia?
Epstein-Barr virus
Hairy leukoplakia is associated with which underlying medical condition?
HIV infection
True or False? Hairy leukoplakia is described as white painless plaques on the tongue that can be easily scraped off.
False; hairy leukoplakia is characterized by white plaques on the tongue that cannot be scraped off
Which autoimmune blistering skin disorder affects the skin but not the oral mucosa?
Bullous pemphigoid
Which blistering autoimmune skin disorder is most severe?
Pemphigus vulgaris
A patient presents with bullae sparing the oral mucosa. From what disease is she suffering?
Bullous pemphigoid
While in bullous pemphigoid immunoglobulin G antibody is directed against the _____, in pemphigus vulgaris immunoglobulin G antibody is directed against the _____.
Hemidesmosomes (epidermal basement membrane); desmosomes
What is the difference in the appearance of bullous pemphigoid and pemphigus vulgaris on immunofluorescence?
Bullous pemphigoid demonstrates linear immunofluorescence whereas pemphigus vulgaris reveals antibodies around cells of the epidermis in a reticular or netlike pattern
A patient presents with large bullae. On exam, there is epidermal separation on stroking of the lesion. What is this sign called and from what disorder is this patient suffering?
Positive Nikolskys sign; the patient has pemphigus vulgaris
True or False? A positive Nikolskys sign is common in both pemphigus vulgaris and bullous pemphigoid.
False; patients with bullous pemphigoid have a negative Nikolskys sign
Patients with bullous pemphigoid have what cell type within blisters?
Eosinophils
What skin condition is associated with celiac disease?
Dermatitis herpetiformis
Dermatitis herpetiformis is associated with pruritic papules and vesicles and deposits of what at the tips of dermal papillae?
Immunoglobulin A
Which skin disorder presents with multiple types of lesions, including macules, papules, vesicles, and target lesions?
Erythema multiforme
List four causes of erythema multiforme.
Infection (Mycoplasma pneumoniae, HSV), drugs, cancers, and autoimmune disease
Which syndrome is characterized by high fever, bulla formation, necrosis, ulceration of the skin, and a high mortality rate?
Stevens-Johnson syndrome
Stevens-Johnson syndrome usually has what etiology?
Drug reaction
A more severe form of Steven-Johnson syndrome is known as what?
Toxic epidermal necrolysis
Which skin disorder involves pruritic, purple, and polygonal papules?
Lichen planus
What infectious disease is associated with lichen planus?
Hepatitis C
Which skin disorder is described as small, rough, erythematous, or brownish papules?
Actinic keratosis
What kind of lesion is actinic keratosis: malignant, benign, or premalignant?
Premalignant
Name two clinical conditions that are associated with acanthosis nigricans.
Hyperlipidemia (ie., from Cushings disease, diabetes) and hyperinsulinemia
Erythema nodosum is characterized by inflammatory lesions of subcutaneous fat located on which part of the body?
Anterior shins
With what disease processes is erythema nodosum associated?
Coccidioidomycosis, histoplasmosis, tuberculosis, leprosy, streptococcal infections, sarcoidosis
What skin disorder is characterized by a herald patch followed by a Christmas tree distribution of lesions?
Pityriasis rosea
Describe the appearance and course of pityriasis rosea.
Multiple papular eruptions with spontaneous remissions
In what stage of life is strawberry hemangioma seen?
During the first few weeks of life
At what age does strawberry hemangioma typically regress?
5 to 8 years of age
At what age do cherry hemangiomas appear?
30 to 49 years of age
Cherry hemangiomas _____ (do/do not) regress spontaneously.
Do not
Squamous cell carcinoma is associated with what environmental exposures?
Sunlight and arsenic
Where does squamous cell carcinoma most commonly occur?
Hands and face
Does squamous cell carcinoma metastasize rarely or commonly?
Rarely
What is the histopathologic finding in squamous cell carcinoma?
Keratin pearls
What is a common precursor lesion to squamous cell carcinoma?
Actinic keratosis
What is the gross appearance of squamous cell carcinoma?
Ulcerative red lesion
Squamous cell carcinoma is associated with what chronic condition?
Associated with chronic draining sinuses in the skin
Keratoacanthoma is a variant of squamous cell carcinoma with what growth pattern?
Rapid growth (4-6 weeks) and spontaneous regression (4-8 weeks)
Where are basal cell carcinomas most commonly found?
On sun-exposed areas of the body
Basal cell tumors have _____ nuclei seen on histology.
Palisading
Basal cell carcinomas are locally invasive but almost never _____.
Metastasize
What is the gross appearance of basal cell carcinomas?
Pearly papules
A bald male patient presents with a lesion on the crown of his head. It has rolled edges with central ulceration. What type of cancer does he have?
Basal cell tumor
What aspect of a melanoma correlates best with the risk of metastases?
Depth of invasion
What lesion is a precursor of melanoma?
Dysplastic nevus (atypical mole)
What tumor marker is associated with melanoma?
S-100
Describe the physical appearance of melanoma on exam.
Dark with irregular borders
