Idiopathic linear IgA bullous dermatosis: prognostic factors based on a case-series of 72 adults

Summary

Background

Linear IgA bullous dermatosis (LABD) is a clinically and immunologically heterogeneous, subepidermal, autoimmune blistering disease (AIBD), whose long-term evolution is poorly described.

Objectives

To investigate the clinical and immunological characteristics, follow-up and prognostic factors of adult idiopathic LABD.

Methods

This retrospective study, conducted in our AIBD Referral Center, included adults, diagnosed between 1995 and 2012, with idiopathic LABD, defined as pure or predominant IgA deposits by direct immunofluorescence. Clinical, histological and immunological findings were collected from charts. Secondarily, standard histology was systematically reviewed, and indirect immunofluorescence (IIF) on salt-split skin (SSS) and immunoblots on amniotic membrane extracts using anti-IgA secondary antibodies (IgA-IB) were performed, when biopsies and sera obtained at diagnosis were available. Prognostic factors for complete remission (CR) were sought with univariate and multivariate analyses.

Results

Among the 72 patients (median age 54 years) included, 60% had mucous membrane (MM) involvement. IgA-IIF–SSS was positive for 21/35 patients tested; 17 had epidermal and dermal labelings. Immunoelectron microscopy on 31 patients' biopsies labeled lamina lucida (LL, 26%), lamina densa (23%), anchoring-fibril zone (AFz, 19%) and LL+AFz (23%). Among 34 IgA-IBs, 22 were positive, mostly for LAD-1/LABD97 (44%) and full-length BP180 (33%). Median follow-up was 39 months. Twenty-four (36%) patients achieved sustained CR, 19 (29%) relapsed and 35% had chronic disease. CR was significantly associated with age >70 years or no MM involvement. No prognostic immunological factor was identified.

Conclusion

LABD patients <70 years old and with MM involvement are at risk for chronic evolution.

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