This is a rare blistering disease. It is an autoimmune subepidermal blistering disease characterized by an autoantibody directed against a 450kd epidermal antigen. It is a member of the plectin family.
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PATHOGENESIS CHARACTERIZATION Autoantibody directed against 450 kd protein
CLINICAL OR GROSS VARIANT CHARACTERIZATION
A case of subepidermal blister disease associated with autoantibody against 450 kD protein.
Fujiwara S, Shinkai H, Takayasu S, Owaribe K, Tsukita S, Kageshita T.
Department of Dermatology, Medical College of Oita, Japan.
J Dermatol 1992 Oct;19(10):610-3 Abstract quote
We report a case of subepidermal blister disease with eruptions distributed on the trunk and extremities. The blisters regressed after administration of corticosteroid and cyclophosphamide. An immunoblot analysis revealed that the patient's serum reacted exclusively with a 450-kD epidermal polypeptide. It did not react with a 230-kD or a 180-kD bullous pemphigoid antigen.
The immunofluorescence study showed the patient's serum reacted with the basement membrane zone of human skin.
J Invest Dermatol 1996;106:1125-1130
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Last Updated June 15, 2005
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