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Sweet Syndrome — SCE Dermatology MCQ

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HardConnective Tissue & VasculitisSweet SyndromeSCE Dermatology

A man with chronic lymphocytic leukaemia develops fever and painful violaceous plaques with pseudovesiculation on the dorsal hands. Biopsy shows papillary dermal oedema and a dense neutrophilic infiltrate without primary leukocytoclastic vasculitis. Which diagnosis is most likely?

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Reveal the answer and explanation

Correct answer: E — Neutrophilic dermatosis of the dorsal hands, representing Sweet syndrome

Explanation lettering: D = shown as A · A = shown as B · E = shown as D · B = shown as E

B is correct. Painful febrile pseudovesicular plaques, haematological disease and a dense dermal neutrophilic infiltrate without primary vasculitis support Sweet syndrome; localisation to the dorsal hands is termed neutrophilic dermatosis of the dorsal hands. Secondary vascular damage may occur in intense lesions but is not the defining process. Leukocytoclastic vasculitis requires vessel-wall injury, erythema elevatum diutinum is a chronic vasculitic/fibrosing papular disorder, multicentric reticulohistiocytosis is histiocytic, and dermatitis herpetiformis is intensely pruritic with granular IgA. Infection should be excluded before immunosuppression because its clinical mimic can be consequential.

Reference: DermNet Sweet syndrome: https://dermnetnz.org/topics/acute-febrile-neutrophilic-dermatosis