A clinical and serological study of linear IgA bullous dermatosis without linear immunoglobulin deposition other than IgA at the basement membrane zone in direct immunofluorescence

Summary

Background

Linear IgA bullous dermatosis (LABD) is a heterogeneous disease. Different diagnostic criteria have been used in different reports.

Objectives

We aimed to reappraise the characteristic features of LABD with only IgA deposition at the basement membrane zone (BMZ) in direct immunofluorescence (DIF).

Methods

We retrospectively collected 101 patients who had a) blisters on the skin and/or mucous membrane, b) subepidermal blisters in a biopsy specimen, and c) linear IgA deposition along BMZ with/without linear C3 deposition at the BMZ in DIF from our archival records from January 1, 1996, through December 31, 2014. Most patients were referred for serological evaluation. Patients who showed concurrent linear IgG and/or IgM deposition at the BMZ in DIF were excluded. Clinical manifestations and serological findings were analyzed.

Results

Heterogeneity of autoantigens in LABD was confirmed in this study. Fifty-four (53.5%) of 101 patients had IgG antibodies detected by either indirect immunofluorescence, immunoblotting (IB), or enzyme-linked immunosorbent assays (ELISA). No statistical difference in clinical manifestations was found between the IgG antibody-possessing group and remaining group.

Conclusion

An association of IgG anti-BMZ antibodies with LABD may increase if new IgG IB or ELISA becomes available. Consensus for diagnostic criteria for LABD is desired for prospective data storage although it may be arbitrary.

This article is protected by copyright. All rights reserved.

http://ift.tt/2hhFCYW