History

Usually, the patient with Wells syndrome (eosinophilic cellulitis) 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 cellulitis-like eruption occurs. The clinical presentation may also include annular plaques, vesicles and bullae (bullous Wells syndrome associated with non-Hodgkin lymphoma^[37]), and urticaria.^[38]

Systemic symptoms, including asthma, arthralgia, and fever, may be evident, although they usually do not occur. Although long-term sequelae usually do not result, reticular pigmentation and scarring alopecia may occur. Rarely, Wells syndrome is associated with life-threatening diseases such leukemia and lymphoma.^{[24, 39, 40]}However, at this point, determining if these are coincidental findings or real associations is impossible.

Physical Examination

The lesions in patients with Wells syndrome (eosinophilic cellulitis) progress over a few days to become large, indurated plaques of edema and erythema, with violaceous edges and no collar. 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 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.

Differential Diagnoses

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Media Gallery

A superficial and deep perivascular and interstitial inflammatory pattern. Courtesy of DermNet New Zealand (https://www.dermnetnz.org/assets/Uploads/pathology/t/wellsfigure2.jpg).
Superficial and deep perivascular and interstitial inflammatory pattern extending into the subcutaneous tissue. Courtesy of DermNet New Zealand (https://www.dermnetnz.org/assets/Uploads/pathology/t/wellsfigure3.jpg).

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Author

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Professor of Pediatrics, Professor of Medicine, Rutgers New Jersey Medical School

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, New York Academy of Medicine, Royal College of Physicians of Edinburgh, Sigma Xi, The Scientific Research Honor Society

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, Sigma Xi, The Scientific Research Honor Society

Disclosure: Received honoraria from Medicis for review panel membership; Received honoraria from Triax for review panel membership.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Acknowledgements

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, Northside 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.

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: Lippincott Williams Wilkins Royalty Textbook editor; DLA Piper Consulting fee Consulting

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.

Wells Syndrome Clinical Presentation

History

Physical Examination

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