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.

Figure 1

The direct immunofluorescence examination demonstrates a continous linear IgA deposition along the basement membrane zone in a patient with linear IgA bullous dermatosis.

Figure 2

(A) Vesiculobullous skin lesions with erosion and crusting limited to the back of an adult patient with linear IgA bullous dermatosis. (B) Arciform vesiculobullous lesions in a “cluster of jewels” configuration in an adult patient with linear IgA bullous dermatosis.

Figure 3

(A) Tense vesiculobullous lesions in the palmar region in an adult patient with linear IgA bullous dermatosis. (B) Erosions and tense bullous lesions on the oral mucosa of an adult patient with linear IgA bullous dermatosis.