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COPD Exacerbation — ABIM Board MCQ

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HardRespiratory MedicineCOPD ExacerbationABIM Board

A 67-year-old with newly diagnosed myelodysplastic syndrome develops fever and exquisitely tender erythematous plaques on the face, neck, and upper limbs. Cultures are negative. Biopsy shows dense papillary-dermal neutrophils and marked edema without primary leukocytoclastic vasculitis. What is the diagnosis?

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Correct answer: A — Sweet syndrome linked to the underlying myelodysplastic syndrome

The best answer is “Sweet syndrome linked to the underlying myelodysplastic syndrome”. Fever, abrupt tender plaques, neutrophilic dermal inflammation, and absence of primary vasculitis establish acute febrile neutrophilic dermatosis, or Sweet syndrome. Its association with myelodysplastic syndrome and acute myeloid leukemia is important and may parallel hematologic activity. Leukocytoclastic vasculitis requires vessel-wall injury, erythema multiforme has target lesions, and pyoderma gangrenosum typically ulcerates and can worsen with surgical trauma.

Reference: Sweet syndrome: a comprehensive review: https://pmc.ncbi.nlm.nih.gov/articles/PMC7752049/