Journal List > J Korean Rheum Assoc > v.15(1) > 1003646

TOOLS
Kim, Chung, Park, Park, Choe, Park, Kim, and Kim: Bullous Eosinophilic Cellulitis (Wells' Syndrome) in a Patient with Churg-Strauss Syndrome: A Case Report and Review of Literatures
Original Article

J Korean Rheum Assoc 2008;15(1):48-53.

Published online: 20 March 2008

DOI: https://doi.org/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

Kyung-Chan Kim, M.D., Hyun Chung, M.D.∗, Joon Soo Park, M.D.∗, Jae Bok Park, M.D.∗∗, Jung-Yoon Choe, M.D., Sung-Hoon Park, M.D., Ji-Young Kim, M.D., Seong-Kyu Kim, M.D.

Department of Internal Medicine, Catholic University of Daegu School of Medicine, Daegu, Korea

∗Department of Dermatology, Catholic University of Daegu School of Medicine, Daegu, Korea

∗∗Department of Pathology, Catholic University of Daegu School of Medicine, Daegu, Korea

Received 10 October 2007       Accepted 30 October 2007

Copyright © 2008 The Korean Rheumatism Association

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

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.

Keywords: Wells' syndrome, Eosinophilic cellulitis, Churg-Strauss syndrome

References

1. Wells GC. Recurrent granulomatous dermatitis with eosinophilia. Trans St Johns Hosp Dermatol Soc. 1971; 57:46–56.
2. Schuttelaar ML, Jonkman MF. Bullous eosinophilic cellulitis (Wells' syndrome) associated with Churg-Strauss syndrome. J Eur Acad Dermatol Venereol. 2003; 17:91–3.
crossref
3. Aberer W, Konrad K, Wolff K. Wells' syndrome is a distinctive disease entity and not a histologic diagnosis. J Am Acad Dermatol. 1988; 18:105–14.
crossref
4. Dijkstra JW, Bergfeld WF, Steck WD, Tuthill RJ. Eosinophilic cellulitis associated with urticaria. A report of two cases. J Am Acad Dermatol. 1986; 14:32–8.
5. Schorr WF, Tauscheck AL, Dickson KB, Melski JW. Eosinophilic cellulitis (Wells' syndrome): histologic and clinical features in arthropod bite reactions. J Am Acad Dermatol. 1984; 11:1043–9.
6. Hirsch K, Ludwig RJ, Wolter M, Zollner TM, Hardt K, Kaufmann R, et al. Eosinophilic cellulitis (Wells' syndrome) associated with colon carcinoma. J Dtsch Dermatol Ges. 2005; 3:530–1.
7. 이운하, 최정철, 전덕규. 식도암 환자에서 발생한 Wells 증후군 1예. 대한피부과학회지. 2000; 43:1130–2.
8. Lee SC, Shin SS, Lee JB, Won YH. Wells syndrome associated with Churg-Strauss syndrome. J Am Acad Dermatol. 2000; 43:556–7.
crossref
9. 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(Supple 44):41.
10. 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. Arthritis Rheum. 1990; 33:1094–100.
11. Pagnoux C, Guilpain P, Guillevin L. Churg-Strauss syndrome. Curr Opin Rheumatol. 2007; 19:25–32.
crossref
12. Strauss L, Churg J, Zak FG. Cutaneous lesions of allergic granulomatosis; a histopathologic study. J Invest Dermatol. 1951; 17:349–59.
13. Herr H, Koh JK. Eosinophilic cellulitis (Wells' syndrome) successfully treated with low-dose cyclosporine. J Korean Med Sci. 2001; 16:664–8.
crossref
14. 이정훈, 전희대, 이성렬, 박영립, 이종석, 황규왕. 전신 PUVA로 치료한 Wells 증후군 1예. 대한피부과학회 지. 2000; 38:949–54.
15. 김은경, 박찬금, 이중달. 호산구성 봉와직염의 임상 및 병리 조직학적 연구. 대한병리학회지. 1995; 29:334–42.

Fig. 1.
Multiple bullous skin lesions on erythematous plaque were noted on the foot.
jkra-15-48f1.tif
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).
jkra-15-48f2.tif
Fig. 3.
Among dermal collagen bundles, there are collections of eosinophils with eosinophilic granules, so called "flame figures" (H&E, ×400).
jkra-15-48f3.tif
Table 1.
Characteristics in Wells' syndrome with Churg-Strauss syndrome
Lee, et al (8) Schuttelaar, et al (2) Govoni, et al (9) Present case
Age/sex 43/female 55/male 43/male 47/male
ACR classification criteria for CSS      
Asthma + + + +
Peripheral WBC (20,800/mm3) WBC (17,000/mm3) WBC (13,200/mm3) WBC (19,700/mm3)
eosinophilia with 55% eosinophils with 34% eosinophils s with 33% eosinophils s with 65% eosinophils
Peripheral Perineural vasculitis with Mononeuritis Mononeuritis Peroneal axonal
neuropathy loss of myelinated fibers multiplex multiplex neuropathy
Transient lung ? ? ? +
infiltrate      
Paranasal sinus NS Nasal polyp Hypertrophic polyposis Paranasal sinusitis
abnormality      
Extravascular NS + NS NS
eosinophils      
in vessel      
ANCA Positive p-ANCA Positive p-ANCA Negative p-ANCA Positive p-ANCA
IgE level 2,422 IU/mL 1,260 E/mL Normal ＞2,000 IU/mL
Involved site(s) Hand, forearm Leg, arm Hand, foot Foot
Treatment Steroid, CySP Steroid, CySP Steroid Steroid
regimens      

ACR: American Collegue of Rheumatology, CSS: Churg-Strauss syndrome, WBC: white blood cell count, p-ANCA: perinuclear anti-neutrophilic cytoplasmic antibody, CySP: cyclophosphamide, NS: no specific description

TOOLS
Similar articles