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

Wells' Syndrome Associated with Churg-Strauss Syndrome

Affiliations
  • 1Department of Dermatology and Cutaneous Biology Research Institute, Yonsei University College of Medicine, Korea.
  • 2Yeouido Oracle Cosmetic & Dermatologic Surgery Clinic, Seoul, Korea. iris980@hanmail.net

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

  • 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.

  • 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).


Reference

1. Wells GC, Smith NP. Eosinophilic cellulitis. Br J Dermatol. 1979. 100:101–109.
Article
2. Schuttelaar ML, Jonkman MF. Bullous eosinophilic cellulitis (Well's syndrome) associated with Churg-Strauss syndrome. J Eur Acad Dermatol Venereol. 2003. 17:91–93.
Article
3. Lee SC, Shin SS, Lee JB, Won YH. Wells syndrome associated with Churg-Strauss syndrome. J Am Acad Dermatol. 2000. 43:556–557.
Article
4. Govoni M, Colina M, Cavazzini L, Trotta F. Churg-Strauss syndrome and Wells syndrome: coincidence or pathogenetic association? A new case report. Clin Exp Rheumatol. 2007. 25:1 Suppl 44. S41.
5. Fujimoto N, Wakabayashi M, Kato T, Nishio C, Tanaka T. Wells syndrome associated with Churg-Strauss syndrome. Clin Exp Dermatol. 2011. 36:46–48.
6. Masi AT, Hunder GG, Lie JT, Michel BA, Bloch DA, Arend WP, et al. The American College of Rheumatology 1990 criteria for the classification of Churg-Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum. 1990. 33:1094–1100.
Article
7. Katoulis AC, Bozi E, Samara M, Kalogeromitros D, Panayiotides I, Stavrianeas NG. Idiopathic bullous eosinophilic cellulitis (Well's syndrome). Clin Exp Dermatol. 2009. 34:e375–e376.
Article
8. Caputo R, Marzano AV, Vezzoli P, Lunardon L. Wells syndrome in adults and children: a report of 19 cases. Arch Dermatol. 2006. 142:1157–1161.
9. Gilliam AE, Bruckner AL, Howard RM, Lee BP, Wu S, Frieden IJ. Bullous "cellulitis" with eosinophilia: case report and review of Well's syndrome in childhood. Pediatrics. 2005. 116:e149–e155.
Article
10. Churg J, Strauss L. Allergic granulomatosis, allergic angiitis, and periarteritis nodosa. Am J Pathol. 1951. 27:277–301.
11. Kallenberg CG. Churg-Strauss syndrome: just one disease entity. Arthritis Rheum. 2005. 52:2589–2593.
Article
Full Text Links
  • AD
Actions
Cited
CITED
export Copy
Close
Share
  • Twitter
  • Facebook
Similar articles
Copyright © 2023 by Korean Association of Medical Journal Editors. All rights reserved.     E-mail: koreamed@kamje.or.kr