eMedicine Specialties > Dermatology > Reactive & Inflammatory Dermatoses

Wells Syndrome (Eosinophilic Cellulitis)

Author: Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Coauthor(s): Justin Brown, MD, Dermatologist, The Dermatology Group
Contributor Information and Disclosures

Updated: Apr 22, 2009

Introduction

Background

In 1971, George Wells first described this syndrome as a recurrent granulomatous dermatitis with eosinophilia.1 Wells and Smith renamed it eosinophilic cellulitis in 1979.2

Eosinophilic cellulitis (Wells syndrome) is an uncommon condition of unknown etiology. The presentation usually involves a mildly pruritic or tender cellulitislike eruption with typical histologic features characterized by edema, flame figures, and a marked infiltrate of eosinophils in the dermis.3 Papular and nodular eruptions at the clinical presentation have also been reported.4,5 The condition can recur and may be preceded by a pruritic papular eruption. Although Wells syndrome is usually sporadic, some familial cases have been reported.

One study showed the successive occurrence of vasculitis, Wells syndrome, and Sweet syndrome in a patient. This finding suggests that an overlap between these diseases exists.6 Another report describes a dominant syndrome consisting of eosinophilic cellulitis, mental retardation, and abnormal body habitus in one family.7

Pathophysiology

The etiology is unknown. At least some cases may represent hypersensitivity to an arthropod bite or sting. A dermal infiltrate of histiocytes, eosinophils, and eosinophilic granules occurs between collagen bundles, which forms the classic flame figures. The eosinophilic infiltrate is almost always restricted to the epidermis and the dermis, but it has also been found in the subcutaneous tissue and the underlying muscle. The location of the infiltrate is correlated with the different clinical features.

In one study, immunophenotyping of peripheral T cells revealed an increased proportion of CD3+ and CD4+ T cells.8 These lymphocytes spontaneously release significant amounts of interleukin 5 (IL-5); this finding suggests that activated T cells may be involved in the pathogenesis of blood and tissue eosinophilia. The eosinophils then degranulate in the dermis, causing edema and inflammation.9

With immunofluorescent stains, eosinophil major basic protein is identified in the granules of the flame figures. On electron microscopy, the collagen fibers are intact; this finding suggests that an initial degeneration of collagen is not a factor in initiating the formation of flame figures.

Eosinophilic cellulitis may be due to drugs, various infections, and, possibly, nonhematological malignancies as trigger events.10,11 Wells syndrome has also been reported to occur in patients with hypereosinophilic syndrome and Churg-Strauss syndrome.

Frequency

United States

Wells syndrome is rare.

International

Only about 80 cases have been reported internationally, including domestically.

Mortality/Morbidity

Although long-term sequelae usually do not result, reticular pigmentation and scarring alopecia may occur.

Race

Wells syndrome can occur in persons of any race.

Sex

No sexual predilection is reported.

Age

Wells syndrome usually affects adults, but it has been known to occur in children.12,13 In one case series of 19 patients, the classic plaque-type presentation was the most common variant found in children, while the annular granuloma–like variant was the most common variant in adults.14

Clinical

History

  • Usually, the patient complains of pruritus or a burning sensation, which is followed by erythema and edema.
  • The clinical presentation can vary widely and may include the following:
    • Annular plaques
    • Vesicles and bullae - Bullous Wells syndrome associated with non-Hodgkin lymphoma15
    • Urticaria16
    • Edema
    • Papules or nodules
  • Typically, a tender or mildly pruritic cellulitislike eruption occurs.
  • Occasionally, papular and nodular eruptions may be seen first.
  • Systemic symptoms, including asthma, arthralgia, and fever, may be evident, although they usually do not occur.

Physical

  • The lesions progress over a few days to become large, indurated plaques of edema and erythema, with violaceous edges and no calor.
  • The lesions may last for several weeks, but they gradually darken from bright red to slate blue.
  • Complete resolution with no scarring is typical, although scarring alopecia may occur.
  • The plaques can occur anywhere on the skin, and they may be solitary or multiple.
  • Plaques on the affected areas are known to recur, and vesiculobullae may develop over the surface.
  • The clinical features seem to depend on the location of the infiltrates in the dermis. This observation suggests that a spectrum of eosinophilic dermatoses occurs in Wells syndrome.

Causes

  • The etiology is unknown. Wells syndrome is usually sporadic, but some familial cases have been described.
  • Suggested precipitating factors include the following:

More on Wells Syndrome (Eosinophilic Cellulitis)

Overview: Wells Syndrome (Eosinophilic Cellulitis)
Differential Diagnoses & Workup: Wells Syndrome (Eosinophilic Cellulitis)
Treatment & Medication: Wells Syndrome (Eosinophilic Cellulitis)
Follow-up: Wells Syndrome (Eosinophilic Cellulitis)
References
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References

  1. Wells GC. Recurrent granulomatous dermatitis with eosinophilia. Trans St Johns Hosp Dermatol Soc. 1971;57(1):46-56. [Medline].

  2. Wells GC, Smith NP. Eosinophilic cellulitis. Br J Dermatol. Jan 1979;100(1):101-9. [Medline].

  3. Brehmer-Andersson E, Kaaman T, Skog E, Frithz A. The histopathogenesis of the flame figure in Wells' syndrome based on five cases. Acta Derm Venereol. 1986;66(3):213-9. [Medline].

  4. Ghislain PD, Van Eeckhout P. Eosinophilic cellulitis of papulonodular presentation (Wells' syndrome). J Eur Acad Dermatol Venereol. Mar 2005;19(2):226-7. [Medline].

  5. Holme SA, McHenry P. Nodular presentation of eosinophilic cellulitis (Wells' syndrome). Clin Exp Dermatol. Nov 2001;26(8):677-9. [Medline].

  6. Consigny S, Courville P, Young P, et al. [Histological and clinical forms of the eosinophilic cellulitis]. Ann Dermatol Venereol. Mar 2001;128(3 Pt 1):213-6. [Medline].

  7. Davis MD, Brown AC, Blackston RD, et al. Familial eosinophilic cellulitis, dysmorphic habitus, and mental retardation. J Am Acad Dermatol. Jun 1998;38(6 Pt 1):919-28. [Medline].

  8. Plotz SG, Abeck D, Behrendt H, Simon HU, Ring J. [Eosinophilic cellulitis (Wells syndrome)]. Hautarzt. Mar 2000;51(3):182-6. [Medline].

  9. Espana A, Sanz ML, Sola J, Gil P. Wells' syndrome (eosinophilic cellulitis): correlation between clinical activity, eosinophil levels, eosinophil cation protein and interleukin-5. Br J Dermatol. Jan 1999;140(1):127-30. [Medline].

  10. Kaufmann D, Pichler W, Beer JH. Severe episode of high fever with rash, lymphadenopathy, neutropenia, and eosinophilia after minocycline therapy for acne. Arch Intern Med. Sep 12 1994;154(17):1983-4. [Medline].

  11. Hirsch K, Ludwig RJ, Wolter M, et al. Eosinophilic cellulitis (Wells' syndrome) associated with colon carcinoma. J Dtsch Dermatol Ges. Jul 2005;3(7):530-1. [Medline].

  12. Anderson CR, Jenkins D, Tron V, Prendiville JS. Wells' syndrome in childhood: case report and review of the literature. J Am Acad Dermatol. Nov 1995;33(5 Pt 2):857-64. [Medline].

  13. Nielsen T, Schmidt H, Sogaard H. Eosinophilic cellulitis. (Well's syndrome) in a child. Arch Dermatol. Jul 1981;117(7):427-9. [Medline].

  14. Caputo R, Marzano AV, Vezzoli P, Lunardon L. Wells syndrome in adults and children: a report of 19 cases. Arch Dermatol. Sep 2006;142(9):1157-61. [Medline].

  15. Spinelli M, Frigerio E, Cozzi A, Garutti C, Garavaglia MC, Altomare G. Bullous Wells' syndrome associated with non-Hodgkin's lymphocytic lymphoma. Acta Derm Venereol. 2008;88(5):530-1. [Medline].

  16. Odia SG, Purschel W, Worret WI, Rakoski J. Hypereosinophilic cellulitis(Wells' syndrome) resembling urticaria. Acta Derm Venerol (Ljubljana). 1994;6:193-195.

  17. Hurni MA, Gerbig AW, Braathen LR, Hunziker T. Toxocariasis and Wells' syndrome: a causal relationship?. Dermatology. 1997;195(4):325-8. [Medline].

  18. Bassukas ID, Gaitanis G, Zioga A, Boboyianni C, Stergiopoulou C. Febrile "migrating" eosinophilic cellulitis with hepatosplenomegaly: adult toxocariasis - a case report. Cases J. Nov 28 2008;1(1):356. [Medline].

  19. Tsuda S, Tanaka K, Miyasato M, Nakama T, Sasai Y. Eosinophilic cellulitis (Wells' syndrome) associated with ascariasis. Acta Derm Venereol. Jul 1994;74(4):292-4. [Medline].

  20. van den Hoogenband HM. Eosinophilic cellulitis as a result of onchocerciasis. Clin Exp Dermatol. Jul 1983;8(4):405-8. [Medline].

  21. Falagas ME, Vergidis PI. Narrative review: diseases that masquerade as infectious cellulitis. Ann Intern Med. Jan 4 2005;142(1):47-55. [Medline].

  22. Leiferman KM, Peters MS. Reflections on eosinophils and flame figures: where there's smoke there's not necessarily Wells syndrome. Arch Dermatol. Sep 2006;142(9):1215-8. [Medline].

  23. Moossavi M, Mehregan DR. Wells' syndrome: a clinical and histopathologic review of seven cases. Int J Dermatol. Jan 2003;42(1):62-7. [Medline].

  24. Stern JB, Sobel HJ, Rotchford JP. Wells' syndrome: is there collagen damage in the flame figures?. J Cutan Pathol. Dec 1984;11(6):501-5. [Medline].

  25. Peters MS, Schroeter AL, Gleich GJ. Immunofluorescence identification of eosinophil granule major basic protein in the flame figures of Wells' syndrome. Br J Dermatol. Aug 1983;109(2):141-8. [Medline].

  26. Aberer W, Konrad K, Wolff K. Wells' syndrome is a distinctive disease entity and not a histologic diagnosis. J Am Acad Dermatol. Jan 1988;18(1 Pt 1):105-14. [Medline].

  27. Herr H, Koh JK. Eosinophilic cellulitis (Wells' syndrome) successfully treated with low-dose cyclosporine. J Korean Med Sci. Oct 2001;16(5):664-8. [Medline].

  28. Mutasim DF, Cooper CH. A case of Wells' syndrome in a patient with lymphocytic lymphoma. Geriatr Dermatol. 1996;4(1):11-14.

  29. Brown J, Schwartz RA. Wells' syndrome (eosinophilic cellulitis). Cesko-Slovenska Dermatol. 2002;77:261-263.

  30. Newton JA, Greaves MW. Eosinophilic cellulitis (Well's syndrome) with florid histological changes. Clin Exp Dermatol. Sep 1988;13(5):318-20. [Medline].

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Further Reading

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Keywords

recurrent granulomatous dermatitis with eosinophilia, Wells' syndrome, Well's syndrome

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Contributor Information and Disclosures

Author

Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi
Disclosure: Nothing to disclose.

Coauthor(s)

Justin Brown, MD, Dermatologist, The Dermatology Group
Justin Brown, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Society for MOHS Surgery, and Sigma Xi
Disclosure: medicis Honoraria Review panel membership; triax Honoraria Review panel membership

Medical Editor

Takeji Nishikawa, MD, Emeritus Professor, Department of Dermatology, Keio University School of Medicine; Director, Samoncho Dermatology Clinic; Managing Director, The Waksman Foundation of Japan Inc
Disclosure: Nothing to disclose.

Pharmacy Editor

David F Butler, MD, Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic
David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Managing Editor

Rosalie Elenitsas, MD, Herman Beerman Associate Professor of Dermatology, University of Pennsylvania School of Medicine; Director, Penn Cutaneous Pathology Services, Department of Dermatology, University of Pennsylvania Health System
Rosalie Elenitsas, MD is a member of the following medical societies: American Academy of Dermatology and American Society of Dermatopathology
Disclosure: Nothing to disclose.

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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