HLA antigen familial study in complete Behçet's syndrome affecting three sisters.

Complete Androgen Insensitivity Syndrome in Three Sisters

Close association of HLA-B51 and B52 in Israeli patients with Behçet's syndrome.

Epidemiological data, family history, clinical data, and HLA typing were studied in three groups of patients with Behçet's syndrome: six Israeli Ashkenazi Jews, 29 non-Ashkenazi Jews, and three Israeli Arabs. HLA-B51 and B52 were present in 24/38 (63%) and 8/38 (21%), respectively, of the patients compared with 13/151 (9%) of the control group for both cases, a relative risk of 18.2 and 2.8 res...

Behçet's syndrome in Scotland.

We present the clinical details and HLA typing of 15 Celtic Caucasian patients (four male, 11 female) with Behçet's syndrome (International Study Group criteria). The males affected were younger than the affected females, and three of these males had severe uveal involvement. Two of the 15 patients had the A2 Bw6 Dr4 haplotype but this did not confer family penetrance. Eight had gastrointestina...

A study of familial occurrence of Behçet's disease with and without ocular lesions.

PURPOSE To examine retrospectively the features of Behçet's disease patients with familial occurrence and make a comparison between familial Behçet's patients with ocular lesions and those without ocular lesions. METHODS We sent questionnaires about Behçet's disease patients with familial occurrence to 114 hospitals that reported treating such patients in previous nationwide hospital surveys,...

Analysis of Human Leukocyte Antigen class II Gene Polymorphism in Iranian Patients with Papillon-Lefevre Syndrome: a Family Study

Background: Papillon-Lefevre syndrome (PLS) is a rare autosomal recessive disorder characterized by palmoplantar hyperkeratosis and early development of aggressive pe-riodontitis. Although cathepsin C (CTSC) gene mutations have been established in about 70-80% of PLS patients, it is assumed that the patients may have dysfunctioning of immune defense mechanisms. Objective: To assess the associat...