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.

Keywords: Autoimmune Diseases; Linear IgA Bullous Dermatosis; Skin Diseases; Vesiculobullous.