title: 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?
abstract: Neonatal lupus erythematosus (NLE) is an uncommon disease that is manifested by cutaneous lesions, cardiac conduction defects, or both, that appear in utero or shortly after birth. In approximately 95% of patients, anti-Ro antibody (Ro[SS-A]) has been identified and has become the serologic marker for NLE. Since 1987 there have been four reported cases of Ro- and anti-La antibody (La[SS-B])-negative, U1RNP antibody-positive, NLE. Our affected twin, as well as all other infants with U1RNP-positive NLE, had cutaneous lesions similar to those in Ro-positive NLE, although they lacked systemic abnormalities, including cardiac conduction defects. HLA typing of mothers with infants with U1RNP-positive NLE revealed the presence of HLA-DR4, DQw1, or DQw3 phenotypes. Our typing confirms these findings. As with Ro-positive NLE, no distinct HLA associations were demonstrated in the infants. Unlike Ro-positive mothers, all mothers with a U1RNP-positive infant with NLE had connective tissue disease at the time of the diagnosis and had a different spectrum of disease. We describe the clinical, serologic, and immunogenetic findings in the first reported case of U1RNP-positive NLE in dizygotic twins in whom the NLE disease expression was discordant.
authors.affiliation: Department of Dermatology, State University of New York-Health Science Center at Brooklyn 11203, USA.
authors.forename: B A
authors.initials: BA
authors.lastname: Solomon
authors.forename: T A
authors.initials: TA
authors.lastname: Laude
authors.forename: A R
authors.initials: AR
authors.lastname: Shalita
mesh_terms: D000328:Adult; D000974:Antibodies, Antinuclear / Q000032:analysis; D004200:Diseases in Twins; D005260:Female; D006680:HLA Antigens / Q000032:analysis; D006801:Humans; D007231:Infant, Newborn; D008178:Lupus Erythematosus, Cutaneous / Q000151:congenital* / Q000235:genetics*; D008180:Lupus Erythematosus, Systemic / Q000235:genetics; D008297:Male; D011247:Pregnancy; D011248:Pregnancy Complications; D017412:Ribonucleoprotein, U1 Small Nuclear / Q000032:analysis*; D014429:Twins, Dizygotic
chemical_list: D000974:Antibodies, Antinuclear; D006680:HLA Antigens; D017412:Ribonucleoprotein, U1 Small Nuclear; C035356:SS-A antibodies; C035357:SS-B antibodies