HLA antigen familial study in complete Behçet's syndrome affecting three sisters.
Behçet's disease is a multisystemic disease affecting most organs. Although a tendency towards an association with a certain genetic type and with HLA-B51 is suspected, the incidence of several siblings with Behçet's disease in a single family is rare. A family, in which three sisters were affected with Behçet's disease, uveitis being the most severe manifestation, was studied. In this family all siblings were B51 positive. Only the female siblings, however, with a positive identical HLA phenotype: A2, A11, B51, B44, Cw6, Cw5, DR4, DRw13, DRw53, DRw52, DQw7, DQw6, developed the disease symptoms, whereas none of the male siblings was affected.
Villanueva
J L
JL
Service of Internal Medicine, Hospital Regional Universitario Reina Sofia, Cordoba, Spain.
Gonzalez-Dominguez
J
J
Gonzalez-Fernandez
R
R
Prada
J L
JL
Peña
J
J
Solana
R
R
D000328:Adult; D001528:Behcet Syndrome / Q000276:immunology* / Q000473:pathology; D005192:Family Health; D005260:Female; D005831:Genital Diseases, Female / Q000473:pathology; D006680:HLA Antigens / Q000032:analysis*; D006801:Humans; D008297:Male; D009059:Mouth Diseases / Q000473:pathology; D010375:Pedigree; D012737:Sex Factors; D014456:Ulcer / Q000473:pathology; D014605:Uveitis / Q000473:pathology
D006680:HLA Antigens