[Systemic lupus erythematosus in a woman with primary 47XXX hypogonadism].

The authors report the case of a 26-year old woman who presented with systemic lupus erythematosus remarkable for its numerous visceral localizations, for the presence of several antinuclear antibodies and for the unusually high serum levels of IgG and IgA. This woman also had primary hypogonadism with ovarian and uterine atrophy, poorly developed secondary sex characteristics and a 47XXX karyotype without mosaic. This case, which may be compared to the association between systemic lupus erythematosus and Klinefelter 47XXX syndrome, suggests a relationship between sex hormones and autoimmune pathology and a possible role of chromosome X in the regulation of immunity.