Va. Venning et F. Wojnarowska, LACK OF PREDICTIVE FACTORS FOR THE CLINICAL COURSE OF BULLOUS PEMPHIGOID, Journal of the American Academy of Dermatology, 26(4), 1992, pp. 585-589
Background: Bullous pemphigoid is a clinically heterogeneous disease a
lthough little is known of the factors affecting its course and outcom
e. Objective: Our purpose was to document the clinical course, outcome
s, 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 pem
phigoid (mean follow-up 3 years 2 months). To identify factors predict
ive of outcome, 16 patients with "good prognosis bullous pemphigoid" (
no systemic treatment or in remission within 2 years) were compared wi
th 12 patients with recurrent disease requiring maintenance therapy wh
o still needed treatment after 3 years or longer. Remission was define
d as 3 months free of lesions, without systemic treatment. Results: Th
e disease duration varied from 9 weeks to 17 years (estimated median t
reatment 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 5
0%. 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 predi
ctive of disease duration. Conclusion: Despite the heterogeneity of th
e clinical course and duration of bullous pemphigoid, no predictive fa
ctors are recognized.