Juvenile Systemic Lupus Erythematosus associated with Klinefelter's syndrome: A case report.

We present the first reported case of juvenile Systemic Lupus Erythematosus with Klinefelter's syndrome in a 14-year-old Iranian boy who had leg ulcers and arthritis. He had low level of testosterone accompanied with hypergonadotropic hypogonadism. This case emphasizes the importance of two X chromosomes as a risk factor for Systemic Lupus Erythematosus in women and men with Klinefelter's syndrome (47, XXY).