A subepidermal blistering disease with histopathological features of dermatitis herpetiformis and immunofluorescence characteristics of bullous pemphigoid: a novel subepidermal blistering disease or a variant of bullous pemphigoid?
A 64-year-old man presented with a bullous eruption which clinically and histopathologically resembled dermatitis herpetiformis. However, direct immunofluorescence analysis showed IgG deposits at the basement membrane zone, indicating a relationship with bullous pemphigoid or epidermolysis bullosa acquisita. Indirect immunofluorescence studies on salt-split skin showed binding of IgG mainly on the dermal side of the blister. Immunoblot analysis revealed a novel 200 kDa dermal antigen that could be associated with a major pathogen in this blistering disease. The histopathological similarity to dermatitis herpetiformis and the immunofluorescence findings indicating bullous pemphigoid or epidermolysis bullosa acquisita seem typical of a distinct subepidermal blistering disease characterized by this 200 kDa antigen. However, the pathogenetic role of autoantibodies against this antigen should be further elucidated before confirming whether this case represents a novel subepidermal blistering disease or a special variant of bullous pemphigoid.
Salmhofer
W
W
Department of Dermatology, University of Graz, Austria.
Kawahara
Y
Y
Soyer
H P
HP
Kerl
H
H
Nishikawa
T
T
Hashimoto
T
T
D001324:Autoantigens / Q000032:analysis; D001485:Basement Membrane / Q000276:immunology; D003874:Dermatitis Herpetiformis / Q000473:pathology*; D003937:Diagnosis, Differential; D005455:Fluorescent Antibody Technique; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis; D008297:Male; D008875:Middle Aged; D010391:Pemphigoid, Bullous / Q000145:classification / Q000276:immunology; D012872:Skin Diseases, Vesiculobullous / Q000276:immunology / Q000473:pathology*
D001324:Autoantigens; D007074:Immunoglobulin G