Ann Dermatol.  2011 Nov;23(4):497-500.

Wells' Syndrome Associated with Churg-Strauss Syndrome

• Lee SH
• Roh MR
• Jee H
• Chung KY
• Jung JY

Abstract

Churg-Strauss syndrome (CSS) is a multisystem granulomatous vasculitis that is characterized by peripheral eosinophilia and the infiltration of eosinophils into systemic organs. The skin lesions of CSS consist mainly of palpable purpura and nodules. Wells' syndrome (WS) is a rare inflammatory dermatosis that is associated with recurrent granulomatous dermatitis and eosinophilia. Since these two diseases are rare, any overlap between them is very unusual. Herein, we report a patient with CSS, who initially presented a skin eruption of erythematous urticarial-plaques, vesicles, and blisters. Upon biopsy, the histology of these plaques indicated eosinophilic infiltration and "flame figures" within the dermis, which was consistent with a diagnosis of WS. Although the association between WS and CSS that was observed in our patient may be purely coincidental, it could also suggest a common pathogenetic background of these two distinct diseases, as both share several many common features.

Keyword

Churg-Strauss syndrome; Flame figures; Wells' syndrome

MeSH Terms

Biopsy
Blister
Cellulitis
Churg-Strauss Syndrome
Dermatitis
Dermis
Eosinophilia
Eosinophils
Humans
Purpura
Skin
Skin Diseases
Vasculitis
Cellulitis
Eosinophilia

Figure

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  Fig. 1 (A) Both forearms contained multiple tense bullae based on erythematous patch with secondary yellowish crusts. (B) Multiple erythematous edematous annular plaques on the trunk, with some central darkening.

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  Fig. 2 (A) Multiple intraepidermal vesicles and subepidermal blisters with underlying perivenular and interstitial inflammatory cell infiltrations in the superficial and deep dermis (H&E, ×40). (B) Perivenular and interstitial infiltration of lymphocytes and eosinophils forming "flame figures" (H&E, ×400).

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