Acquired junctional epidermolysis bullosa associated with IgG autoantibodies to the beta subunit of laminin-5.
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 beta 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 beta 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.
Kirtschig
G
G
Department of Dermatology, University Hospital, Marburg, Germany.
Caux
F
F
McMillan
J R
JR
Bedane
C
C
Aberdam
D
D
Ortonne
J P
JP
Eady
R A
RA
Prost
C
C
D000368:Aged; D001323:Autoantibodies / Q000378:metabolism; D001327:Autoimmune Diseases / Q000276:immunology*; D016107:Epidermolysis Bullosa Acquisita / Q000276:immunology*; D016109:Epidermolysis Bullosa, Junctional / Q000276:immunology*; D019084:Fluorescent Antibody Technique, Indirect; D006801:Humans; D015151:Immunoblotting; D007074:Immunoglobulin G / Q000378:metabolism*; D007797:Laminin / Q000737:chemistry / Q000276:immunology*; D008297:Male; D016253:Microscopy, Immunoelectron
D001323:Autoantibodies; D007074:Immunoglobulin G; D007797:Laminin