Lack of predictive factors for the clinical course of bullous pemphigoid.

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.

• Authors: V A Venning, F Wojnarowska
• Journal: Journal of the American Academy of Dermatology (J Am Acad Dermatol) Vol. 26 Issue 4 Pg. 585-9 (Apr 1992) ISSN: 0190-9622 [Print] United States
• PMID: 1597545 (Publication Type: Journal Article)

Chemical References

• Dapsone
• Prednisolone
• Azathioprine

Topics

• Aged
• Aged, 80 and over
• Autoimmune Diseases (drug therapy, mortality)
• Azathioprine (therapeutic use)
• Dapsone (therapeutic use)
• Drug Therapy, Combination
• Female
• Follow-Up Studies
• Humans
• Male
• Middle Aged
• Pemphigoid, Bullous (drug therapy, mortality)
• Prednisolone (therapeutic use)
• Prognosis
• Recurrence
• Remission Induction
• Treatment Outcome