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Dermatology · Skin tumours: Sezary syndrome
Sample itemA 65-year-old has diffuse erythroderma, severe itch, generalised lymphadenopathy and cells with cerebriform nuclei in peripheral blood. What is the diagnosis?
Original practice item, not a recalled exam question. Nothing here is recorded.
The answer and the reasoning
A. Sezary syndrome
Sezary syndrome is a leukaemic variant of cutaneous T-cell lymphoma, with erythroderma, lymphadenopathy and circulating malignant CD4 T cells with cerebriform nuclei. Prognosis is poorer than for mycosis fungoides. Flow cytometry and T-cell receptor clonality help confirm it.
Option by option
A. Sezary syndrome (correct)
Correct. Erythroderma with circulating cerebriform cells is Sezary syndrome.
B. Pityriasis rubra pilaris
PRP shows islands of sparing and orange keratoderma.
C. Psoriatic erythroderma
Psoriatic erythroderma has a history of psoriasis and no circulating malignant cells.
D. Atopic erythroderma
Atopic erythroderma has a history of atopy without circulating atypical lymphocytes.
Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.
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