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Inflammatory epidermolysis bullosa acquisita with coexistent IgA antibodies to plectin
Author(s) -
Buijsrogge J. J. A.,
De Jong M. C. J. M.,
Meijer H. J.,
Dijk F.,
Jonkman M. F.,
Pas H. H.
Publication year - 2005
Publication title -
clinical and experimental dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.587
H-Index - 78
eISSN - 1365-2230
pISSN - 0307-6938
DOI - 10.1111/j.1365-2230.2005.01854.x
Subject(s) - epidermolysis bullosa acquisita , plectin , epidermolysis bullosa , immunoelectron microscopy , immunofluorescence , antibody , pathology , epidermolysis bullosa simplex , medicine , autoantibody , immunology , biology , intermediate filament , genetics , cytoskeleton , cell
Summary We present a case of inflammatory epidermolysis bullosa acquisita (EBA) with IgA antibodies to plectin. Analysis of lesional skin biopsies by electron microscopy revealed the split level to be in the sublamina densa zone, corresponding to the diagnosis of EBA. Direct immunofluorescence of perilesional skin demonstrated u‐serrated depositions of IgG and IgA that under immunoelectron microcopy were shown to be located in the sublamina densa. In contrast, indirect immunofluorescence on salt‐split skin revealed circulating IgA antibodies that stained the roof rather than the floor of the blister. Immunoblotting showed these serum antibodies to be directed to the cytoplasmic hemidesmosomal antigen plectin. The antiplectin specificity of these antibodies was confirmed by ‘knockout’ immunofluorescence analysis; the serum IgA did not bind to skin sections of a patient with plectin‐deficient epidermolysis bullosa. To our knowledge, this case demonstrates for the first time the existence of IgA antibodies against plectin.