Neonatal lupus erythematosus: Discordant disease expression of U1RNP-positive antibodies in fraternal twins—Is this a subset of neonatal lupus erythematosus or a new distinct syndrome?

摘要: Neonatal lupus erythematosus (NLE) is an uncommon disease that manifested by cutaneous lesions, cardiac conduction defects, or both, appear in utero shortly after birth. In approximately 95% of patients, anti-Ro antibody (Ro[SS-A]) has been identified and become the serologic marker for NLE. Since 1987 there have four reported cases Ro- anti-La (La[SS-B])-negative, U1RNP antibody-positive, Our affected twin, as well all other infants with U1RNP-positive NLE, had lesions similar to those Ro-positive although they lacked systemic abnormalities, including defects. HLA typing mothers NLE revealed presence HLA-DR4, DQw1, DQw3 phenotypes. confirms these findings. As no distinct associations were demonstrated infants. Unlike mothers, a infant connective tissue at time diagnosis different spectrum disease. We describe clinical, serologic, immunogenetic findings first case dizygotic twins whom expression was discordant.