[Senear-Usher syndrome: acquired hemophilia caused by the occurrence of a factor VIII inhibitor].
A 25-year-old woman who had suffered from pemphigus for 2 years developed widespread, scalded skin-like lesions after stopping an immunosuppressive treatment. Histological and immunofluorescence studies confirmed the diagnosis of pemphigus erythematosus. Apart from IgG pemphigus antibodies, granular deposits for IgM at the dermoepidermal junction and circulating antinuclear IgM-antibodies could be detected. Despite of restarting an immunosuppressive regime, the patient developed severe hemophilia due to the occurrence of a factor VIII antiglobulin. Its possible immunopathologic basis is discussed.
D000328:Adult; D001323:Autoantibodies / Q000032:analysis; D005169:Factor VIII / Q000037:antagonists & inhibitors* / Q000276:immunology; D005260:Female; D006467:Hemophilia A / Q000209:etiology*; D006801:Humans; D010392:Pemphigus / Q000150:complications* / Q000276:immunology
D001323:Autoantibodies; D005169:Factor VIII
Senear-Usher-Syndrom: Begleithämophilie durch das Auftreten eines Faktor-VIII-Hemmkörpers.