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Bullous Pemphigoid Associated with Shy‐Drager Syndrome
Author(s) -
Okazaki Aiko,
Iida Takashi,
Muramatsu Tsutomu,
Shirai Toshihiko,
Murata Kenya,
Takayanagi Tetsuya
Publication year - 1998
Publication title -
the journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.9
H-Index - 65
eISSN - 1346-8138
pISSN - 0385-2407
DOI - 10.1111/j.1346-8138.1998.tb02436.x
Subject(s) - bullous pemphigoid , dermatology , medicine , pemphigoid , immunology , antibody
We report a patient with Shy‐Drager syndrome who developed multiple tense blisters mainly on the extremities. Circulating anti‐basement membrane zone autoantibodies were detected by the indirect immunofluorescence method. Immunoblot analysis using normal human epidermal extracts demonstrated that this patient's serum reacted only with 230 kD bullous pemphigoid antigen (BPAG1). Concerning the pathoetiology of the association of bullous pemphigoid and Shy‐Drager syndrome, we discuss a sequence similarity between BPAG1 and dystonin, a candidate gene for dystonia musculorum.