V A Venning1, F Wojnarowska. 1. Department of Dermatology, Slade Hospital, Oxford, United Kingdom.
Abstract
BACKGROUND: Bullous pemphigoid is a clinically heterogeneous disease although little is known of the factors affecting its course and outcome. OBJECTIVE: Our purpose was to document the clinical course, outcomes, and causes of death in treated bullous pemphigoid and to determine the predictive factors affecting outcome. METHODS: The clinical course was documented in 82 patients with immunologically proven bullous pemphigoid (mean follow-up 3 years 2 months). To identify factors predictive of outcome, 16 patients with "good prognosis bullous pemphigoid" (no systemic treatment or in remission within 2 years) were compared with 12 patients with recurrent disease requiring maintenance therapy who still needed treatment after 3 years or longer. Remission was defined as 3 months free of lesions, without systemic treatment. RESULTS: The disease duration varied from 9 weeks to 17 years (estimated median treatment time 2 years 1 month). Of patients followed up for at least 2 years, 30% achieved remission and by 3 years the remission rate was 50%. Two patients had a subsequent relapse (9%). The mortality rate at 1 year was 19%, and treatment was believed to be contributory in seven deaths. No clinical, immunologic, or immunogenetic factors were predictive of disease duration. CONCLUSION: Despite the heterogeneity of the clinical course and duration of bullous pemphigoid, no predictive factors are recognized.
BACKGROUND: Bullous pemphigoid is a clinically heterogeneous disease although little is known of the factors affecting its course and outcome. OBJECTIVE: Our purpose was to document the clinical course, outcomes, and causes of death in treated bullous pemphigoid and to determine the predictive factors affecting outcome. METHODS: The clinical course was documented in 82 patients with immunologically proven bullous pemphigoid (mean follow-up 3 years 2 months). To identify factors predictive of outcome, 16 patients with "good prognosis bullous pemphigoid" (no systemic treatment or in remission within 2 years) were compared with 12 patients with recurrent disease requiring maintenance therapy who still needed treatment after 3 years or longer. Remission was defined as 3 months free of lesions, without systemic treatment. RESULTS: The disease duration varied from 9 weeks to 17 years (estimated median treatment time 2 years 1 month). Of patients followed up for at least 2 years, 30% achieved remission and by 3 years the remission rate was 50%. Two patients had a subsequent relapse (9%). The mortality rate at 1 year was 19%, and treatment was believed to be contributory in seven deaths. No clinical, immunologic, or immunogenetic factors were predictive of disease duration. CONCLUSION: Despite the heterogeneity of the clinical course and duration of bullous pemphigoid, no predictive factors are recognized.
Authors: Katherine E Brick; Chad H Weaver; Christine M Lohse; Mark R Pittelkow; Julia S Lehman; Michael J Camilleri; Mustafa Al-Hashimi; Carilyn N Wieland Journal: J Am Acad Dermatol Date: 2014-04-03 Impact factor: 11.527