UNUSUAL MANIFESTATIONS OF TYPE-1 AUTOIMMUNE POLYENDOCRINOPATHY

We present a family with five members affected by Type 1 autoimmune po lyendocrinopathy. All patients had chronic mucocutaneous candidiasis a nd dental abnormalities, Four patients had ocular abnormalities, four had hypoparathyroidism, and three had Addison's disease. The family wa s unusual in that all four affected females had premature ovarian fail ure. The ocular abnormalities included two patients with subcapsular l ens opacities, one patient with asymptomatic corneal opacities, and on e patient with severe bilateral iridocyclitis with cataract formation, One patient had pernicious anaemia and one had insulin dependent diab etes mellitus, All patients were negative on repeated occasions for or gan specific and non-organ specific autoantibodies. Lymphocyte studies were performed in four patients. A deficiency of T suppressor cells w as found in three and low normal levels were present in the fourth sug gesting that the syndrome may be due to a defect in suppressor T cells .