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Pruritic, Annular Plaques With Pustules on a Middle-aged Man

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A Japanese man in his 50s presented with a 6-month history of worsening pruritic hand dermatitis that progressed to involve his face, trunk, upper extremities, and feet. He had emigrated from Japan more than 10 years earlier but had no history of recent travel. He took no medications, had no significant medical or surgical history, and denied personal and family history of atopy. While he worked as a biomedical researcher, he denied any caustic exposures. On physical examination, his face, trunk, upper extremities, palms, and soles were marked by 1- to 2-mm erythematous papules and pustules coalescing into large, partially indurated and polycyclic plaques with centrifugal extension and occasional central clearing (Figure, A and B). Treatment with potent topical corticosteroids was ineffective. Punch biopsies of lesions on the shoulder (Figure, C) and foot (Figure, D) were performed.

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C. Eosinophilic pustular folliculitis (EPF)

Histopathologic analysis of a follicular papule on the shoulder demonstrated a perivascular and periadnexal eosinophilic and lymphocytic infiltrate concentrated at the follicular isthmus and sebaceous glands (Figure, C). A plaque on the glabrous plantar foot showed eosinophilic spongiosis and subcorneal and intraepidermal pustules (Figure, D). Tissue stains and cultures were negative for bacterial, mycobacterial, and fungal organisms. Neither lesional tissue nor blood demonstrated a monoclonal T-cell population on T-cell receptor (TCR) gene rearrangement studies. Flow cytometry did not detect an atypical B- or T-cell population in the blood. Laboratory workup was notable for an elevated absolute eosinophil count of 1100/μL (1.1 × 109/L). Complete blood cell counts, results of a comprehensive metabolic panel, and lactate dehydrogenase levels were otherwise unremarkable. Findings of human immunodeficiency virus (HIV) testing were negative. Collectively, these findings were compatible with a diagnosis of classic EPF, or Ofuji disease.

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Article Information

Corresponding Author: Xiaolong (Alan) Zhou, MD, Department of Dermatology, Northwestern University Feinberg School of Medicine, 676 N St Clair St, Arkes 1600, Chicago, IL 60611 (alan.zhou@northwestern.edu).

Published Online: February 13, 2019. doi:10.1001/jamadermatol.2018.5197

Conflict of Interest Disclosures: None reported.

Additional Contributions: We thank the patient for granting permission to publish this information.

References
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