Linear IgA bullous dermatosis: 32 years of experience

Abstract

Background

Linear IgA bullous dermatosis (LABD) is an uncommon disease with only a few reported studies in large series with long follow-up periods.

Objectives

To evaluate the clinical presentation, immunopathological features, management, and disease course in LABD patients.

Methods

Data including demographics, clinical features, histopathological and immunofluorescence findings of LABD patients, in addition to the preferred treatments and responses to treatments were evaluated.

Results

Among 26 patients diagnosed with LABD, 17 (65.4%) were female. The mean age was 40.3 ± 22.4 (6‒80) years of whom 21 were adults. The most common mucosal involvement was oral (n = 9, 34.6%). Continuous linear IgA deposition was present on the basement membrane zone of all patients in addition to C3 (n = 13), IgG (n = 9), IgM (n = 4), and fibrinogen (n = 4). Three patients were lost to follow-up without any treatment. Dapsone was the treatment of choice in most (n = 21, 91.3%) patients in addition to systemic corticosteroids (n = 17), azathioprine (n = 3), tetracycline and nicotinamide (n = 2). Complete and partial remissions were achieved in 11 (47.8%) and 12 (52.2%) patients, respectively, in a mean follow-up period of 45.9 ± 43.9 (3‒158) months. Furthermore, 17 patients were still under treatment at the end of the follow-up period.

Study limitations

Retrospective study conducted in a single center.

Conclusions

LABD may occur at two separate peaks, one in the second and the other in the sixth decade of life with a female predominance. Other immunoglobulins may be associated with dominant IgA antibody deposition and the most commonly used therapeutic option for LABD patients was oral dapsone.