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Bullous Wells' Syndrome: Case Report and Systematic Review.

Giulia Ciccarese1, Giorgia Sbarra2, Giovanni Liguori2

  • 1Dermatology Unit, Department of Medical of Surgical Sciences, University of Foggia, Viale L. Pinto, 1, 71122 Foggia, Italy.

Journal of Clinical Medicine
|December 11, 2025
PubMed
Summary

Bullous Wells' syndrome (BWS) is a rare skin condition characterized by itchy blisters. This study analyzed 28 cases, finding corticosteroids effective, but also highlighting potential links to other diseases and new treatments.

Keywords:
bullous Wells’ syndromeeosinophilic cellulitisitchy bullous lesions

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Area of Science:

  • Dermatology
  • Immunology

Background:

  • Wells' syndrome (WS) is a rare cutaneous disorder of unknown cause.
  • Bullous Wells' syndrome (BWS) is a rare subtype presenting with itchy bullous lesions and erythematous plaques.

Purpose of the Study:

  • To describe a case of BWS in a patient with chronic lymphocytic leukemia.
  • To systematically review the literature on BWS, analyzing clinical, laboratory, histological features, and treatments.

Main Methods:

  • Systematic literature review using Ovid MEDLINE, PubMed, and EMBASE.
  • Search terms included "bullous Wells' syndrome," "eosinophilic cellulitis," and "bullous eosinophilic dermatitis."
  • Analysis of 28 case reports, including the presented case.

Main Results:

  • The study analyzed 28 patients (primarily adult females, median age 44.92 years).
  • Common findings included blood eosinophilia, eosinophilic-neutrophilic dermal infiltrate, and bullae often preceded by urticarial plaques, primarily on extremities.
  • Potential triggers include medications, insect bites, malignancies, and autoimmune/infectious diseases; systemic steroids are first-line treatment, with Mepolizumab showing efficacy in refractory cases.

Conclusions:

  • Diagnosing BWS is challenging due to its rarity, varied presentation, and differential diagnoses.
  • Integrating clinical, laboratory, and histopathological findings is crucial for accurate diagnosis.
  • Investigating potential underlying neoplastic, autoimmune, or infectious conditions is important for optimal patient management, even if a causal link is not always evident.