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Acquired junctional epidermolysis bullosa associated with IgG autoantibodies to the β subunit of laminin‐5
Author(s) -
Kirtschig,
Caux,
McMillan,
Bedane,
Daniel Aberdam,
Ortonne,
Eady,
Prost
Publication year - 1998
Publication title -
british journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.304
H-Index - 179
eISSN - 1365-2133
pISSN - 0007-0963
DOI - 10.1046/j.1365-2133.1998.02038.x
Subject(s) - junctional epidermolysis bullosa (veterinary medicine) , hemidesmosome , immunoelectron microscopy , lamina densa , lamina lucida , epidermolysis bullosa acquisita , epidermolysis bullosa , immunogold labelling , autoantibody , laminin , pathology , anchoring fibrils , immunofluorescence , basement membrane , medicine , biology , antibody , immunology , microbiology and biotechnology , immunohistochemistry , ultrastructure , basal lamina , extracellular matrix
We report the case of a 72‐year‐old man with clinical features resembling those of non‐lethal junctional epidermolysis bullosa associated with IgG autoantibodies to the β chain of laminin‐5. The patient presented with a sudden onset of blistering and severe fragility of the skin and mucous membranes resulting in atrophic scars. Electron microscopy showed that the blistering arose in the lamina lucida. Indirect immunofluorescence indicated that the autoantibodies bound to the dermal side of 1 mol/L NaCl‐split skin, and both direct and indirect immunoelectron microscopy demonstrated antibody binding to the lamina densa. Postembedding immunogold electron microscopy also revealed labelling in the lamina lucida beneath the hemidesmosomes. On immunoblotting, we found the autoantibodies to comigrate with the β chain of laminin‐5. Following the nomenclature of inherited junctional epidermolysis bullosa with mutations of the laminin‐5 gene, we propose the name acquired junctional epidermolysis bullosa for this newly recognized disease.