Bullous Wells' Syndrome: Case Report and Systematic Review

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.