Wells Syndrome (Eosinophilic Cellulitis) Clinical Presentation
- Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD more...
History
Usually, the patient reports pruritus or a burning sensation, which is followed by erythema and edema. Occasionally, papular and nodular eruptions may be seen first. Typically, a tender or mildly pruritic cellulitislike eruption occurs. Systemic symptoms, including asthma, arthralgia, and fever, may be evident, although they usually do not occur.
The clinical presentation can vary widely and may include the following:
- Annular plaques
- Vesicles and bullae - Bullous Wells syndrome associated with non-Hodgkin lymphoma[16]
- Urticaria[17]
- Edema
- Papules or nodules
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:
- Arthropod bites and stings, including those of the honeybee[18]
- Cutaneous viral infections: There is a possible link between parvoviral infection and Wells syndrome.[19]
- Cutaneous parasitic infestations, including toxocariasis,[20, 21] ascariasis,[22] and onchocerciasis[23]
- Leukemia
- Myeloproliferative disorders
- Fungal infections
- Hypersensitivity reactions to medications or metals, including metallic alloy implants[24]
- Churg-Strauss syndrome: This syndrome has been associated in a few patients.[25] These reports are noteworthy for the presence of bullae and of antineutrophil cytoplasmic antibodies.[26] Only a few other autoimmune diseases have been associated, including a case report of systemic lupus erythematosus.[27]
Wells GC. Recurrent granulomatous dermatitis with eosinophilia. Trans St Johns Hosp Dermatol Soc. 1971;57(1):46-56. [Medline].
Wells GC, Smith NP. Eosinophilic cellulitis. Br J Dermatol. Jan 1979;100(1):101-9. [Medline].
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].
Ghislain PD, Van Eeckhout P. Eosinophilic cellulitis of papulonodular presentation (Wells' syndrome). J Eur Acad Dermatol Venereol. Mar 2005;19(2):226-7. [Medline].
Holme SA, McHenry P. Nodular presentation of eosinophilic cellulitis (Wells' syndrome). Clin Exp Dermatol. Nov 2001;26(8):677-9. [Medline].
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].
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].
Koga C, Sugita K, Kabashima K, Matsuoka H, Nakamura M, Tokura Y. High responses of peripheral lymphocytes to mosquito salivary gland extracts in patients with Wells syndrome. J Am Acad Dermatol. Jul 2010;63(1):160-1. [Medline].
Plotz SG, Abeck D, Behrendt H, Simon HU, Ring J. [Eosinophilic cellulitis (Wells syndrome)]. Hautarzt. Mar 2000;51(3):182-6. [Medline].
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].
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].
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].
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].
Nielsen T, Schmidt H, Sogaard H. Eosinophilic cellulitis. (Well's syndrome) in a child. Arch Dermatol. Jul 1981;117(7):427-9. [Medline].
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].
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].
Odia SG, Purschel W, Worret WI, Rakoski J. Hypereosinophilic cellulitis(Wells' syndrome) resembling urticaria. Acta Derm Venerol (Ljubljana). 1994;6:193-195.
Lin HL, Lin JN, Chen CW, Kuo LC, Lee WC. Eosinophilic cellulitis after honeybee sting. J Formos Med Assoc. Dec 2009;108(12):964-6. [Medline].
Cherng E, McClung AA, Rosenthal HM, Hicks J, Levy ML. Wells' Syndrome Associated with Parvovirus in a 5-Year Old Boy. Pediatr Dermatol. Dec 9 2011;[Medline].
Hurni MA, Gerbig AW, Braathen LR, Hunziker T. Toxocariasis and Wells' syndrome: a causal relationship?. Dermatology. 1997;195(4):325-8. [Medline].
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].
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].
van den Hoogenband HM. Eosinophilic cellulitis as a result of onchocerciasis. Clin Exp Dermatol. Jul 1983;8(4):405-8. [Medline].
Zhu L, Hu D, Wang Q, Hou J, Li M. Diffuse polymorphic eosinophilic cellulitis in a patient with metallic alloy implants: a possible association?. Int J Dermatol. Dec 2011;50(12):1535-7. [Medline].
Lee SH, Roh MR, Jee H, Chung KY, Jung JY. Wells' syndrome associated with churg-strauss syndrome. Ann Dermatol. Nov 2011;23(4):497-500. [Medline]. [Full Text].
Fujimoto N, Wakabayashi M, Kato T, Nishio C, Tanaka T. Wells syndrome associated with Churg–Strauss syndrome. Clin Exp Dermatol. Jan 2011;36(1):46-8. [Medline].
Yin G, Xie Q. Systemic lupus erythematosus associated with Wells' syndrome. Rheumatol Int. Feb 22 2011;[Medline].
Falagas ME, Vergidis PI. Narrative review: diseases that masquerade as infectious cellulitis. Ann Intern Med. Jan 4 2005;142(1):47-55. [Medline].
Shams M, Hudgens J, Lesher JL Jr, Florentino F. Wells' syndrome presenting as a noninfectious bullous cellulitis in a child. Pediatr Dermatol. Mar 2012;29(2):224-6. [Medline].
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].
Moossavi M, Mehregan DR. Wells' syndrome: a clinical and histopathologic review of seven cases. Int J Dermatol. Jan 2003;42(1):62-7. [Medline].
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].
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].
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].
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].
Mutasim DF, Cooper CH. A case of Wells' syndrome in a patient with lymphocytic lymphoma. Geriatr Dermatol. 1996;4(1):11-14.
Brown J, Schwartz RA. Wells' syndrome (eosinophilic cellulitis). Cesko-Slovenska Dermatol. 2002;77:261-263.

