A study of familial lupus erythematosus-like disease and hereditary angio-oedema treated with danazol.
Immunological and genetic studies were performed in a family in which the mother and five siblings had hereditary angio-oedema (HAE). LE cells, antinuclear factors, antibodies to double-stranded DNA and positive direct Coombs' test were not found in any of the subjects. One female sibling had a lupus erythematosus-like illness with skin lesions of the face which responded to danazol treatment. Laboratory evidence of HAE was obtained in the entire kindred and all the siblings shared HLA haplotype AIB7 inherited from the deceased mother.
D000293:Adolescent; D000328:Adult; D000799:Angioedema / Q000150:complications / Q000188:drug therapy / Q000235:genetics*; D003165:Complement System Proteins / Q000032:analysis; D003613:Danazol / Q000627:therapeutic use*; D005148:Facial Dermatoses / Q000150:complications; D005260:Female; D006680:HLA Antigens / Q000032:analysis; D015788:HLA-A1 Antigen; D015793:HLA-B7 Antigen; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000150:complications / Q000188:drug therapy / Q000235:genetics*; D008297:Male; D010375:Pedigree; D011245:Pregnadienes / Q000627:therapeutic use*
D006680:HLA Antigens; D015788:HLA-A1 Antigen; D015793:HLA-B7 Antigen; D011245:Pregnadienes; D003165:Complement System Proteins; D003613:Danazol