Bullous pemphigoid associated with Shy-Drager syndrome.
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.
Department of Dermatology, Nara Medical University, Japan.
A
A
Okazaki
T
T
Iida
T
T
Muramatsu
T
T
Shirai
K
K
Murata
T
T
Takayanagi
D001323:Autoantibodies / Q000032:analysis; D001324:Autoantigens / Q000235:genetics / Q000276:immunology; D001485:Basement Membrane / Q000276:immunology; D002352:Carrier Proteins / Q000235:genetics / Q000276:immunology; D003094:Collagen; D003598:Cytoskeletal Proteins / Q000235:genetics / Q000276:immunology; D000071821:Dystonin; D004817:Epidermis / Q000276:immunology; D019084:Fluorescent Antibody Technique, Indirect; D006801:Humans; D015151:Immunoblotting; D008297:Male; D008875:Middle Aged; D009419:Nerve Tissue Proteins / Q000235:genetics / Q000276:immunology; D024041:Non-Fibrillar Collagens; D010391:Pemphigoid, Bullous / Q000150:complications* / Q000276:immunology; D012791:Shy-Drager Syndrome / Q000150:complications*; D000097805:Collagen Type XVII
D001323:Autoantibodies; D001324:Autoantigens; D002352:Carrier Proteins; D003094:Collagen; D003598:Cytoskeletal Proteins; D000071821:Dystonin; D009419:Nerve Tissue Proteins; D024041:Non-Fibrillar Collagens; D000097805:Collagen Type XVII; C486268:DST protein, human