DISCOID LUPUS-ERYTHEMATOSUS ASSOCIATED WITH A PRIMARY IMMUNODEFICIENCY SYNDROME SHOWING FEATURES OF NON-X-LINKED HYPER-IGM SYNDROME

Hyper-IgM (HIM) syndrome is a rare primary immunodeficiency disorder. Approximately 120 cases have been described in the literature world-wi de. Features of HIM include low serum IgG, a very low IgA with normal or high IgM levels, Autoimmune phenomena are recognized associations b ut connective tissue disorders have so far not been described in HIM p atients. We report the case of a 19-year-old Indian woman with an immu nodeficiency syndrome characteristic of non-X-linked HIM who developed discoid lupus erythematosus. Anti-double-stranded DNA antibodies were negative. Antibodies to extractable nuclear antigens were positive fo r Ro and nRNP, with evidence that they were of both IgG and IgM class. Treatment with hydroxychloroquine and topical steroids was successful .