April 2015 Minnesota Dermatological Society Meeting: Medical Dermatology MOC

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Dermatology Quizzes & Trivia

Questions and Answers
  • 1. 

    A 32 year old female presents with a 10 year history of persistent tender, scaly plaques affecting her scalp and pubic area. Review of systems reveals a long history of polydipsia and polyuria. Skin biopsy shows a dermal and epidermal infiltration of large polygonal histiocytes with eccentric nuclei. What stains are most helpful in confirming the diagnosis? 

    • A.

      A. CD3 and CD8

    • B.

      B. S-100 and MART-1

    • C.

      C. S-100 and CD1a

    • D.

      D. CD163 and CD68

    • E.

      E. CK20 and chromogranin

    Correct Answer
    C. C. S-100 and CD1a
    Explanation
    The clinical presentation of persistent tender, scaly plaques affecting the scalp and pubic area, along with the long history of polydipsia and polyuria, suggests a diagnosis of Langerhans cell histiocytosis (LCH). The histological findings of dermal and epidermal infiltration of large polygonal histiocytes with eccentric nuclei further support this diagnosis. The stains that are most helpful in confirming the diagnosis of LCH are S-100 and CD1a. S-100 is a marker for neural crest-derived cells, including Langerhans cells, while CD1a is a specific marker for Langerhans cells. Therefore, staining for S-100 and CD1a can help confirm the presence of Langerhans cells in the affected tissues.

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  • 2. 

    A 3 year old boy with failure to thrive, progressive hearing loss, polyuria and polydipsia presents with extensive eczematous scaling in a seborrhea-like distribution. Skin pathology shows large abnormal histiocytes with reniform nuclei that stain positive for S100 and CD1a. Furthermore, genetic testing reveals a V600E BRAF mutation. What does he have? 

    • A.

      A. Urticaria pigmentosa

    • B.

      B. Li-Fraumeni syndrome

    • C.

      C. Cowden Syndrome

    • D.

      D. Familial melanoma syndrome

    • E.

      E. Erdheim Chester disease

    Correct Answer
    E. E. Erdheim Chester disease
    Explanation
    The patient's presentation of failure to thrive, progressive hearing loss, polyuria, polydipsia, and extensive eczematous scaling in a seborrhea-like distribution is consistent with Erdheim Chester disease. Skin pathology findings of large abnormal histiocytes with reniform nuclei that stain positive for S100 and CD1a, along with the presence of a V600E BRAF mutation on genetic testing, further support this diagnosis. Erdheim Chester disease is a rare form of non-Langerhans cell histiocytosis that typically presents in adulthood but can also occur in children. It is characterized by the infiltration of histiocytes into various organs, including the skin, bones, and central nervous system.

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  • 3. 

    3. A 57 year old Caucasian male with history of hepatitis C and resultant cirrhosis was complicated by acute renal failure requiring dialysis after transplant. He received Galolinium magnetic resonance cholangiopancreatography during this time and subsequently rapidly developed indurated, erythematous plaques on trunk and extremities.Which immunohistochemical staining pattern would provide most support for the most likely clinical diagnosis? 

    • A.

      A. CD 34 positive lymphocytes

    • B.

      B. CD 34 positive spindle shaped cells

    • C.

      C. CD 134 positive lymphocytes

    • D.

      D. CD 134 positive spindle shaped cells

    • E.

      E. CD 134 negative spindle shaped cells

    Correct Answer
    B. B. CD 34 positive spindle shaped cells
    Explanation
    The most likely clinical diagnosis in this case is nephrogenic systemic fibrosis (NSF), which is a rare condition that occurs in patients with renal insufficiency who have been exposed to gadolinium-based contrast agents. The development of indurated, erythematous plaques on the trunk and extremities is consistent with NSF. CD 34 is a marker for endothelial cells, and in this case, CD 34 positive spindle-shaped cells would provide the most support for the diagnosis of NSF.

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  • 4. 

    A 60 year old Caucasian female developed sudden onset edema, pain and sclerosis with subsequent contractures of bilateral hands and feet. This was accompanied by significant fatigue as well as debilitating polyarthralgia and muscle weakness. ANA shows low titer. Incidentally, an ovarian carcinoma was identified.Which of the following findings best differentiates paraneoplastic Palmar Fasciitis and Polyarthritis Syndrome from Systemic Scleroderma?

    • A.

      A: Presence of joint pains

    • B.

      B: Severe mobility difficulty flexing and extending all fingers

    • C.

      B: Severe mobility difficulty flexing and extending all fingers C: Swelling of dorsum hand

    • D.

      D: Lack of Raynaud’s phenomenon

    • E.

      E: Presence of muscle weakness

    Correct Answer
    D. D: Lack of Raynaud’s phenomenon
    Explanation
    Paraneoplastic Palmar Fasciitis and Polyarthritis Syndrome is characterized by sudden onset edema, pain, and sclerosis with subsequent contractures of the hands and feet. It is often accompanied by fatigue, polyarthralgia, and muscle weakness. In this case, the presence of an ovarian carcinoma is an important clue. The finding that best differentiates this syndrome from Systemic Scleroderma is the lack of Raynaud's phenomenon. Raynaud's phenomenon is a common feature of Systemic Scleroderma, but it is not typically seen in Paraneoplastic Palmar Fasciitis and Polyarthritis Syndrome.

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  • 5. 

    Lenalidomide is a thalidomide derivative that has been shown in select case reports to be efficacious in treating severe, refractory prurigo nodularis. Its side effect profile includes which of the following:

    • A.

      A. Risk for fetal limb defects

    • B.

      B. Thrombocytosis

    • C.

      C. Deep venous thrombosis

    • D.

      D. A & B

    • E.

      E. A & C

    Correct Answer
    E. E. A & C
    Explanation
    Lenalidomide, a derivative of thalidomide, has been found to be effective in treating severe, refractory prurigo nodularis. However, it is important to be aware of its side effect profile. Lenalidomide has been associated with an increased risk for fetal limb defects, making it contraindicated in pregnant women. Additionally, it has been linked to the development of deep venous thrombosis, a potentially serious condition. Therefore, the correct answer is E, as both options A and C accurately describe the side effect profile of lenalidomide.

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  • 6. 

    Which of the following areas are least likely to be affected by the orodynia of burning mouth syndrome?

    • A.

      A. Anterior two thirds of the tongue

    • B.

      B. Buccal mucosa

    • C.

      C. Lower lip

    • D.

      D. Palate

    • E.

      E. Tonsil

    Correct Answer
    B. B. Buccal mucosa
    Explanation
    Buccal mucosa refers to the lining of the cheeks, which is least likely to be affected by the orodynia of burning mouth syndrome. Burning mouth syndrome typically affects the tongue, lips, and palate, causing a burning sensation and discomfort. The tonsils may also be affected in some cases. However, the buccal mucosa is less likely to be affected as it is not directly involved in the primary symptoms of burning mouth syndrome.

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  • 7. 

    Often times, contributing factors are identified in patients with burning mouth syndrome. Which of the following is most likely to contribute to increased symptoms in burning mouth syndrome?

    • A.

      A. Depression

    • B.

      B. Zinc deficiency

    • C.

      C. Thiamine deficiency

    • D.

      D. History of dental caries

    • E.

      E. Gluten sensitive enteropathy

    Correct Answer
    A. A. Depression
    Explanation
    Depression is most likely to contribute to increased symptoms in burning mouth syndrome. Depression can worsen the perception of pain and increase sensitivity to symptoms. It can also affect the body's ability to cope with and manage pain. Additionally, depression can lead to changes in neurotransmitter levels, which can further exacerbate symptoms.

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  • 8. 

    A 15 year old male presents for evaluation of painful calluses on his feet and thick toenails. Further physical examination reveals white patches on the oral mucosa and cystic lesions on the axilla. What is the most likely diagnosis? 

    • A.

      A. Steatocytoma multiplex

    • B.

      B. Pachyonychia congenita treatment is necessary

    • C.

      C. Clouston Syndrome

    • D.

      D. Olmsted syndrome D. Olmsted syndrome

    • E.

      E. Vörner keratoderma

    Correct Answer
    B. B. Pachyonychia congenita treatment is necessary
    Explanation
    The most likely diagnosis in this case is Pachyonychia congenita. This is supported by the presence of painful calluses on the feet, thick toenails, white patches on the oral mucosa, and cystic lesions on the axilla. Pachyonychia congenita is a rare genetic disorder that affects the nails, skin, and mucous membranes. Treatment is necessary to manage the symptoms and improve the patient's quality of life.

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  • 9. 

    A 13 year old boy develops a well demarcated verrucous plaque that grows over a one week period. He lives in Indiana and has animals including a horse, dog, and cat at home. He denies any constitutional symptoms including or any bone pain or hemoptysis. A biopsy of the plaque shows broad based budding yeast forms on GMS. Which of the following is his most likely diagnosis? 

    • A.

      A. Histoplasmosis

    • B.

      B. Mucormycosis

    • C.

      C. Coccidiomycosis

    • D.

      D. Sporotrichosis

    • E.

      E. Candida albicans

    Correct Answer
    A. A. Histoplasmosis
    Explanation
    The patient's presentation of a well-demarcated verrucous plaque that grows rapidly over a short period of time, along with the presence of animals at home, suggests a fungal infection. The biopsy findings of broad-based budding yeast forms on GMS staining are characteristic of Histoplasmosis. Histoplasmosis is caused by the fungus Histoplasma capsulatum, which is commonly found in soil contaminated with bird or bat droppings. The patient's residence in Indiana, known for its high prevalence of histoplasmosis, further supports this diagnosis.

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  • 10. 

    A 28 year old male cook from Southern Minnesota developed a cough productive of bloody sputum and a small, pink papule on his left cheek. Within one month, the papule grew into a 4 cm exophytic nodule with a keratotic crust. At the same time he began to develop multiple small, asymptomatic granulomatous pink papules on his lip, arms, and back. These also began to slowly grow. He originally presented to an emergency department where he was prescribed trimethoprim-sulfamethoxazole for ten days, which despite taking the medication as prescribed, had no effect on his papules and nodules. He had no recent travel history and nooutdoor hobbies. A biopsy of one of the nodules shows broad based budding yeast forms on GMS. What is the following best treatment for this patient? 

    • A.

      A. Amphoteracin B, intravenously

    • B.

      B. Itraconazole 200 mg PO BID

    • C.

      C. Keflex 500 mg PO QID

    • D.

      D. Observation

    • E.

      E. Surgical excision

    Correct Answer
    B. B. Itraconazole 200 mg PO BID
    Explanation
    The patient's presentation with a cough productive of bloody sputum, along with the development of a papule on the cheek that grew into an exophytic nodule with a keratotic crust, suggests a fungal infection. The presence of multiple small granulomatous pink papules on the lip, arms, and back further supports this. The biopsy showing broad-based budding yeast forms on GMS confirms the diagnosis of a fungal infection, specifically sporotrichosis. The treatment of choice for sporotrichosis is itraconazole, making option B the best treatment for this patient.

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  • 11. 

    A 73 year old woman has a several month history of painful upper and lower lip erosions. She endorses five pound unintentional weight loss secondary to pain with eating. She denies intraoral or genital involvement. She denies any changes to her medications or new cosmetic products prior to onset. Her past medical history is significant for hypertension and osteoarthritis. On examination, upper and lower cutaneous lip ulcerations are seen, in addition to faint white linear plaques on her bilateral buccal mucosa. Swab for HSV/VZV is negative.Which of the following is the most likely diagnosis? 

    • A.

      A. Discoid lupus erythematous

    • B.

      B. Actinic cheilitis

    • C.

      C. Lichen planus

    • D.

      D. Contact dermatitis

    • E.

      E. Steven-Johnson syndrome

    Correct Answer
    C. C. Lichen planus
    Explanation
    The patient's presentation of painful lip erosions, weight loss, and faint white linear plaques on the buccal mucosa is consistent with the diagnosis of lichen planus. Lichen planus is a chronic inflammatory condition that can affect the skin, mucous membranes, hair, and nails. In this case, the involvement of the lips and buccal mucosa, along with the absence of intraoral or genital involvement, points towards lichen planus as the most likely diagnosis. The negative HSV/VZV swab also rules out herpes simplex or varicella-zoster virus as the cause. Discoid lupus erythematosus, actinic cheilitis, contact dermatitis, and Steven-Johnson syndrome do not fit the clinical presentation.

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  • 12. 

    Different regimens for treatment of oral lichen planus have been reported. Topical tacrolimus is one such treatment. Which of the following is the most likely side effect of this medication?

    • A.

      A. Folliculitis

    • B.

      B. Erythema

    • C.

      C. Photosensitivity

    • D.

      D. Burning

    • E.

      E. Anaphylaxis

    Correct Answer
    D. D. Burning
    Explanation
    Topical tacrolimus is a medication used for the treatment of oral lichen planus. It is an immunosuppressive agent that works by reducing inflammation in the affected area. The most likely side effect of this medication is a burning sensation. This burning sensation may occur at the site of application and is a common side effect reported by patients using topical tacrolimus. It is important for healthcare providers to inform patients about this potential side effect and to monitor their response to the medication.

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  • 13. 

    A 60 year old male presents with an urticarial eruption, fever, fatigue, and joint pain. Physical examination revealed widespread urticarial plaques, axillary lymphadenopathy and hepatosplenomegaly. Laboratory evaluation revealed a white blood cell count of 25x109/L, an erythrocyte sedimentation rate (ESR) of 118mm/h, and protein electrophoresis revealed an M spike identified as an IgM K protein. Which of the following is an obligate criterion for the diagnosis of this condition?

    • A.

      A. Joint Pain

    • B.

      B. Leukocytosis

    • C.

      C. Elevated ESR

    • D.

      D. Monoclonal IgM

    • E.

      E. Fever

    Correct Answer
    D. D. Monoclonal IgM
    Explanation
    The presence of a monoclonal IgM spike is an obligate criterion for the diagnosis of this condition. This indicates the presence of a monoclonal gammopathy, which is a characteristic finding in certain conditions such as Waldenström macroglobulinemia. The other symptoms and laboratory findings mentioned in the question are associated with this condition but are not obligate criteria for diagnosis.

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  • 14. 

    Which of the following is the treatment of choice for this condition?

    • A.

      A. Antihistamines

    • B.

      B. Intravenous Immunoglobulin

    • C.

      IL-1 receptor antagonists

    • D.

      Dapsone

    • E.

      Systemic corticosteriods

    Correct Answer
    C. IL-1 receptor antagonists
    Explanation
    IL-1 receptor antagonists are the treatment of choice for this condition. This suggests that IL-1 plays a significant role in the pathogenesis of the condition and blocking its receptors can be an effective therapeutic approach. Antihistamines, intravenous immunoglobulin, dapsone, and systemic corticosteroids may have some benefits in certain conditions, but IL-1 receptor antagonists are specifically mentioned as the preferred treatment option.

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  • 15. 

    A five year old female presented for evaluation of diffuse hair loss. She was born with normal lanugo hair; however, at two months of life she shed her hair diffusely. She has remained without terminal hair on the body, scalp, eyelashes or eyebrows. Physical examination was notable complete absence of terminal hair across the scalp, face, and trunk. Dentition and fingernails were normal. Miniature follicles were appreciated on the scalp, cheeks, arms and legs. Genetic testing revealed mutation in the hairless (HR) gene.What does this patient have?

    • A.

      A. Alopecia areata

    • B.

      B. Woolly hair syndrome

    • C.

      C. Congenital atrichia

    • D.

      D. Alopecia universalis

    • E.

      E. Netherton syndrome

    Correct Answer
    C. C. Congenital atrichia
    Explanation
    This patient has congenital atrichia, as evidenced by the complete absence of terminal hair on the body, scalp, eyelashes, and eyebrows. The presence of miniature follicles on the scalp, cheeks, arms, and legs further supports this diagnosis. The mutation in the hairless (HR) gene, revealed by genetic testing, is consistent with congenital atrichia.

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  • 16. 

    Which of the following histopathologic findings are most compatible with this diagnosis?

    • A.

      A. Mild peribulbar, lymphocytic inflammation and miniaturization

    • B.

      B. Absence of normal hair follicle structures with normal sebaceous and eccrine glands.

    • C.

      C. Empty hair shafts surrounded by mild, granulomatous inflammation and loss of surrounding sebaceous glands.

    • D.

      D. Miniaturization of follicular units with fibrous tract remnants..

    • E.

      E. Absence of normal hair follicle structures with normal sebaceous and eccrine glands.

    Correct Answer
    E. E. Absence of normal hair follicle structures with normal sebaceous and eccrine glands.
  • 17. 

    Compared to classic mycosis fungoides, which of the following phenotype is seen most commonly seen in patients with poikilodermatous mycosis fungoides?

    • A.

      A. CD4+

    • B.

      B. CD8+

    • C.

      C. CD20+

    • D.

      D. CD30+

    • E.

      E. CD2+

    Correct Answer
    B. B. CD8+
    Explanation
    Patients with poikilodermatous mycosis fungoides commonly exhibit a phenotype characterized by the presence of CD8+ cells. This suggests that CD8+ T cells play a significant role in the pathogenesis of this variant of mycosis fungoides. The other options (CD4+, CD20+, CD30+, CD2+) are not as commonly associated with poikilodermatous mycosis fungoides.

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  • 18. 

    A 48 year old male presents with a 10 year history of telangiectatic and reticulated thin plaques on his trunk and extremities. They have been persistent, pruritic, and involve 10% of his body surface area. A biopsy is performed and reveals an atypical T-cell infiltrate with a CD8+ phenotype and epidermotropism. He has no systemic complaints. What would be the most appropriate initial therapy for this patient?

    • A.

      A. Acitretin

    • B.

      B. CHOP chemotherapy

    • C.

      C. Narrowband UVB

    • D.

      D. Methotrexate

    • E.

      E. Alpha interferon

    Correct Answer
    C. C. Narrowband UVB
    Explanation
    The most appropriate initial therapy for this patient would be narrowband UVB. The patient's presentation of telangiectatic and reticulated thin plaques on the trunk and extremities, along with persistent pruritus and involvement of 10% of the body surface area, suggests a diagnosis of mycosis fungoides (MF), a type of cutaneous T-cell lymphoma. Narrowband UVB phototherapy is a commonly used treatment for early-stage MF, as it can help to reduce the severity of skin lesions and improve symptoms. The other options, including acitretin, CHOP chemotherapy, methotrexate, and alpha interferon, are not typically used as initial therapies for early-stage MF.

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  • 19. 

    A baby is delivered in a collodion membrane. At early childhood he develops large, dark grey or brownish thick scales covering much of the body surface. Associated ectropion and eclabium are noted. Which of the following is the most likely diagnosis? 

    • A.

      A. Anhidrotic ectodermal dysplasia

    • B.

      B. Lamellar Ichthyosis

    • C.

      C. Netherton syndrome

    • D.

      D. Richner-Hanhart syndrome

    • E.

      E. Olmsted syndrome

    Correct Answer
    B. B. Lamellar Ichthyosis
    Explanation
    The presentation of a baby being delivered in a collodion membrane, followed by the development of large, dark grey or brownish thick scales covering much of the body surface, along with associated ectropion and eclabium, is characteristic of Lamellar Ichthyosis. This is a rare genetic disorder that affects the skin's ability to shed dead skin cells properly, leading to the formation of scales. The other options listed are not associated with this specific presentation.

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  • 20. 

    In a child with autosomal recessive ichthyosis and the TGM1 mutation, what are the histopathological findings most likely demonstrated? 

    • A.

      A. Lichenoid inflammation and basal vacuolar interface changes

    • B.

      B. Orthokeratosis and focal parakeratosis with a normal to thickened granular layer

    • C.

      C. Psudoepitheliomatous hyperplasia with neutrophilic collections in the dermis

    • D.

      D. Regular acanthosis with clear cell change and peppered neutrophils

    • E.

      E. Thickened basement membrane with periadnexal and perivascular inflammation

    Correct Answer
    B. B. Orthokeratosis and focal parakeratosis with a normal to thickened granular layer
  • 21. 

    A 2 year old presented with brittle hair. Scanning electron microsopy showed flattened hair shafts that are twisted along the long axis. Further history revealed that she was recently diagnosed with sensorineural hearing loss. A genetic mutation in the BCS1 gene was found. Which of the following is the most likely diagnosis?

    • A.

      A. Menkes syndrome

    • B.

      B. GRACILE syndrome

    • C.

      C. Netherton syndrome

    • D.

      D. Hay-Wells syndrome

    • E.

      E. Xeroderma pigments

    Correct Answer
    B. B. GRACILE syndrome
    Explanation
    GRACILE syndrome is the most likely diagnosis in this case. GRACILE syndrome is a rare autosomal recessive disorder characterized by growth restriction, aminoaciduria, cholestasis, iron overload, lactacidosis, and early death. It is caused by mutations in the BCS1L gene, which is involved in the assembly of mitochondrial respiratory chain complex III. The symptoms of brittle hair and twisted hair shafts seen in the patient are consistent with the hair abnormalities seen in GRACILE syndrome. The presence of sensorineural hearing loss further supports the diagnosis, as it is a common feature of GRACILE syndrome.

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  • 22. 

    An infant has a history of abnormal hair growth, early developmental delay, lethargy, and seizures. Prior workup includes a mutation in the ATP7A gene. Abnormalities in what trace element are most important in these patients?

    • A.

      A. Copper

    • B.

      B. Iron

    • C.

      C. Manganese

    • D.

      D. Selenium

    • E.

      E. Zinc

    Correct Answer
    A. A. Copper
    Explanation
    The infant's symptoms, such as abnormal hair growth, early developmental delay, lethargy, and seizures, are consistent with Menkes disease, which is caused by a mutation in the ATP7A gene. Menkes disease is a copper metabolism disorder, where there is impaired copper absorption and transport. Copper is an essential trace element that plays a crucial role in various physiological processes, including neurodevelopment. Therefore, abnormalities in copper levels are most important in these patients, explaining why copper is the correct answer.

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  • 23. 

    In subcutaneous panniculitis-like T cell lymphoma, alpha/beta phenotype, which of the following is the most likely histopathology?

    • A.

      A. Adipocytes with an atypical lymphoid infiltrate

    • B.

      B. Lobular panniculitis with neutrophils

    • C.

      C. Septal pannicultis with eosinophils

    • D.

      D. Necrosis of adipocytes

    • E.

      E. Hyalinizing fat necrosis with calcium deposits

    Correct Answer
    A. A. Adipocytes with an atypical lymphoid infiltrate
    Explanation
    The most likely histopathology in subcutaneous panniculitis-like T cell lymphoma, alpha/beta phenotype is adipocytes with an atypical lymphoid infiltrate. This means that there is an abnormal infiltration of lymphoid cells within the adipose tissue. This finding is characteristic of this type of lymphoma and helps differentiate it from other types of panniculitis. Lobular panniculitis with neutrophils, septal panniculitis with eosinophils, necrosis of adipocytes, and hyalinizing fat necrosis with calcium deposits are not typically seen in subcutaneous panniculitis-like T cell lymphoma.

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  • 24. 

    When compared with alpha/beta subcutaneous panniculitis like T cell lymphoma, the gamma-delta subtype is more likely to demonstrate which of the following?

    • A.

      A. Angioinvasion

    • B.

      B. Lack of infiltration of upper dermis and epidermis

    • C.

      C. CD34 positivity

    • D.

      D. Association with hemophagocytic syndrome

    • E.

      E. A & D

    • F.

      F. B & C

    Correct Answer
    E. E. A & D
    Explanation
    The gamma-delta subtype of subcutaneous panniculitis-like T-cell lymphoma is more likely to demonstrate angioinvasion and association with hemophagocytic syndrome. Angioinvasion refers to the invasion of blood vessels by cancer cells, which is more commonly seen in the gamma-delta subtype. Hemophagocytic syndrome is a condition characterized by the overactivation of the immune system, leading to excessive inflammation and destruction of blood cells. This syndrome is also more commonly associated with the gamma-delta subtype of lymphoma.

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  • 25. 

    A 50-year-old male presents to the clinic for evaluation of rapid onset, symmetric and bilateral fibrosis of his extremities. Raynaud’s sign is absent. Laboratory evaluation includes an elevated ESR and eosinophil count,with a normal anti-scl 70 and anti-centromere antibodies. Histopathologic examination of an incisional biopsy shows deep sclerosis including the fascia without eosinophils. Which of the following is the most likely diagnosis? 

    • A.

      A. Porphyria cutanea tarda

    • B.

      B. Systemic sclerosis

    • C.

      C. Eosinophilic fasciitis

    • D.

      D. Discoid lupus

    • E.

      E. Limited sclerosis

    Correct Answer
    C. C. Eosinophilic fasciitis
    Explanation
    The patient's presentation of rapid onset, symmetric and bilateral fibrosis of the extremities, along with an elevated ESR and eosinophil count, suggests eosinophilic fasciitis. The absence of Raynaud's sign and the normal anti-scl 70 and anti-centromere antibodies further support this diagnosis. Histopathologic examination showing deep sclerosis including the fascia without eosinophils is also consistent with eosinophilic fasciitis. Porphyria cutanea tarda is unlikely as it does not typically present with fibrosis. Systemic sclerosis may present with fibrosis, but the absence of Raynaud's sign and the specific histopathologic findings make eosinophilic fasciitis a more likely diagnosis. Discoid lupus and limited sclerosis do not fit the clinical presentation.

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  • 26. 

    Which of the following is the most appropriate initial step in management of eosinophilic fasciitis? 

    • A.

      A. Methotrexate

    • B.

      B. UVA1 UVA1 UVA1 B. UVA1

    • C.

      C. Doxycycline

    • D.

      D.High dose prednisone (1mg/kg)

    • E.

      E. Mycophenolate mofetil

    Correct Answer
    D. D.High dose prednisone (1mg/kg)
    Explanation
    High dose prednisone (1mg/kg) is the most appropriate initial step in the management of eosinophilic fasciitis. Eosinophilic fasciitis is an inflammatory disorder that affects the fascia, causing thickening and fibrosis. Prednisone, a corticosteroid, is the mainstay of treatment for this condition. High dose prednisone is effective in reducing inflammation and improving symptoms. Other options such as methotrexate, UVA1 phototherapy, doxycycline, and mycophenolate mofetil may be used as adjunctive or alternative treatments, but they are not the initial step in management.

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  • 27. 

    Mutations in ATP2A2 gene cause dysregulation of endoplasmic reticulum Ca2+ ATPase (SERCA2), which interferes with intracellular Ca2+ signaling. Loss of suprabasilar cell adhesion (acantholysis) and induction of apoptosis (dyskeratosis) results.  ATP2A2 gene mutation is implicated in which of the following diseases? 

    • A.

      A. Acrokeratosis verruciformis of Hopf

    • B.

      B. Darier disease

    • C.

      C. Hailey-Hailey disease

    • D.

      D. Severe seborrheic dermatitis

    • E.

      E. Transient Acantholytic Dermatosis

    Correct Answer
    B. B. Darier disease
    Explanation
    Mutations in the ATP2A2 gene cause dysregulation of endoplasmic reticulum Ca2+ ATPase (SERCA2), which interferes with intracellular Ca2+ signaling. This disruption in Ca2+ signaling leads to the loss of suprabasilar cell adhesion (acantholysis) and induction of apoptosis (dyskeratosis). Darier disease is a rare genetic skin disorder characterized by the presence of acantholysis and dyskeratosis. Therefore, ATP2A2 gene mutation is implicated in Darier disease.

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  • 28. 

    Mutations in ATP2C1 gene cause dysregulation of Golgi-associated Ca2+ ATPase, which interferes with intracellular Ca2+ signaling. Clinical exam finds flaccid blisters and erosions on the neck and in intertriginous areas, especially the axillae and groin.  Moist malodorous vegetations and fissures can develop. Histologic findings include acantholysis throughout the spinous layer. ATP2C1 gene mutation is implicated in which disease? 

    • A.

      A. Acrokeratosis verruciformis of Hopf

    • B.

      B. Darier disease

    • C.

      C. Hailey-Hailey disease

    • D.

      D. Severe seborrheic dermatitis

    • E.

      E. Transient Acantholytic Dermatosis

    Correct Answer
    C. C. Hailey-Hailey disease
    Explanation
    Mutations in the ATP2C1 gene are known to cause Hailey-Hailey disease. This genetic mutation leads to dysregulation of the Golgi-associated Ca2+ ATPase, which disrupts intracellular calcium signaling. Clinical examination findings, such as flaccid blisters and erosions in specific areas like the neck, axillae, and groin, along with the development of moist malodorous vegetations and fissures, are characteristic of Hailey-Hailey disease. Histologic findings, including acantholysis throughout the spinous layer, further support the diagnosis of Hailey-Hailey disease.

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  • Current Version
  • Mar 22, 2023
    Quiz Edited by
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  • Apr 17, 2014
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    MNDERM
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