Familial Behcet's syndrome

Abstract

Behçet's syndrome is reported in a family of which four generations have so far been affected. The index patient also exhibited an unusual schizo-affective disorder. The histocompatibility antigen haplotype 1-17 was common to four with the disease. Genetic transmission may be responsible for the familial nature of the syndrome and the associated schizophrenia.

Immunopathogenesis of Behçet's Disease

“…The mean age is the same, but the disease seems to be milder in females. Familial BD has been reported (32)(33)(34)(35). This is most probably due to the association of the environmental factor together with the histocompatibility antigen in the same family.…”

Behcet's syndrome: a family study and the elucidation of a genetic role.

“…In a psychiatric study of Behget's syndrome (Epstein et al, 1970) submissiveness, depression, and neuroses were observed. Goolamali et al (1976) described the development of acute schizophrenia with affective features in one of their patients.…”

“…Both viral (Beh9et, 1937;Sezer, 1952Sezer, , 1953Sezer, , 1956Evans et al, 1957;Dudgeon, 1961) and autoimmune (Jensen, 1941;Shimizu et al, 1965) mechanisms have been postulated and familial occurrence has been reported (Fadli and Youssef, 1973;Fowler et al, 1968;Mason and Barnes, 1969;Sezer, 1956). Goolamali et al (1976) presented a 4-generation family study of Beh9et's syndrome associated with psychiatric disorders, and a genetic transmission was postulated. Patients…”

Behcet’s disease

“…A series of reports referred to the familial incidence of the disease, which was especially evident among the pediatric age group [50][51][52][53]. Genetic anticipation was recorded in 5 of 18 families with 40 affected members in two successive generations [54].…”