J Korean Rheum Assoc.  2008 Mar;15(1):48-53. 10.4078/jkra.2008.15.1.48.

Bullous Eosinophilic Cellulitis (Wells' Syndrome) in a Patient with Churg-Strauss Syndrome: A Case Report and Review of Literatures

• Kim KC
• Chung H
• Park JS
• Park JB
• Choe JY
• Park SH
• Kim JY
• Kim SK

• KMID: 1324508
• DOI: http://doi.org/10.4078/jkra.2008.15.1.48

Abstract

Wells' syndrome is an inflammatory dermatosis with associated aberrant eosinophil responses caused by unknown factors. Its histology is characterized by erythematous plaques with "flame figures" in the dermis, which is potentially diagnostic but not pathognomic. Cases of Wells' syndrome in patients with Churg-Strauss syndrome (CSS), which is characterized by antineutrophil cytoplasmic antibody-related necrotizing vasculitis, marked peripheral eosinophilia, and eosinophil tissue infiltrates, have rarely been reported, and the pathogenic association between these two diseases remains undetermined. Differences of clinical and histopathologic features of these two diseases suggest that they are distinct disease entities, even though, in part, they share pathogenic mechanisms. Here we present a new case with Wells' syndrome in a patient with CSS, treated with systemic steroid.

Keyword

Wells' syndrome; Eosinophilic cellulitis; Churg-Strauss syndrome

Figure

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  Fig. 1. Multiple bullous skin lesions on erythematous plaque were noted on the foot.

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  Fig. 2. There is a heavy dermal infiltration of inflammatory cells (A: H&E, ×40), composed of lymphocytes and histiocytes with conspicuous eosinophils (B: H&E, ×200).

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  Fig. 3. Among dermal collagen bundles, there are collections of eosinophils with eosinophilic granules, so called "flame figures" (H&E, ×400).

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