Review Question - QID 109314

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Mycosis Fungoides / Sezary Syndrome Evaluation Diagnosis confirmed with skin biopsy QID: 109314 (M1.ON.17.4799)

QID 109314 (Type "109314" in App Search)

A 58-year-old female presents to her dermatologist complaining of a painful skin rash. She reports that the rash is red, itchy, and peeling. She also reports intermittent fatigue, night sweats, and chills. Her past medical history is notable for hypertension and diabetes mellitus for which she takes lisinopril and metformin. She has previously had two irregular skin lesions removed from her face and neck that eventually were shown to be squamous cell carcinoma. She has a 20 pack-year smoking history and drinks several alcoholic beverages per day. Her temperature is 98.9°F (37.1°C), blood pressure is 140/85 mmHg, pulse is 95/min, and respirations are 18/min. Physical examination reveals a red rash predominantly found on her trunk and thighs, inguinal lymphadenopathy, and hepatosplenomegaly. A notable physical examination finding is shown in Figure A. A biopsy of this patient's skin would most likely reveal which of the following?

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Keratinocyte acantholysis with preserved basal cell attachment

17%

26/149

Atypical keratinocytes with keratin pearls

11%

17/149

Pigmented cells with marked atypia and prominent nucleoli

6/149

Nests of basaloid cells in the dermis with palisades

10/149

Atypical lymphocytes with cerebriform nuclei

56%

84/149

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The patient in this vignette presents with a painful pruritic rash, systemic symptoms (e.g., fatigue and night sweats), lymphadenopathy, and organomegaly suggestive of Sezary syndrome. This condition is characterized by atypical CD4+ T-cells with cerebriform nuclei on histology.

Sezary syndrome is a variant of cutaneous T-cell lymphoma (CTCL) that is characterized by both skin involvement and leukemia (malignant T-cells in the blood). Mycosis fungoides is the more common form of CTCL that is limited to the skin. Histologic analysis may also reveal Pautrier microabscesses within the thickened epidermis.

Figure A demonstrates the characteristic appearance of a skin lesion in Sezary syndrome. These lesions are typically found in non-sun exposed areas and are red, scaling, and pruritic. Illustration A demonstrates the characteristic appearance of Sezary cells – atypical CD4+ T-cells with cerebriform nuclei.

Incorrect Answers:
Answer 1: Pemphigus vulgaris is characterized by keratinocyte acantholysis with preserved basal cell attachment to the dermis.

Answer 2: Squamous cell carcinoma is characterized by atypical keratinocytes with keratin pearls on histology.

Answer 3: Melanoma is characterized by pigmented cells with marked atypia and prominent nucleoli on histology.

Answer 4: Basal cell carcinoma is characterized by nests of basaloid cells in the dermis with palisades on histology.

Bullet Summary:
Sezary syndrome is a form of cutaneous T-cell lymphoma with associated leukemia that is characterized by atypical CD4+ lymphocytes with cerebriform nuclei on histology.

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