[Neonatal lupus erythematosus: case report and review of the literature].
Neonatal lupus erythematosus (NLE) is a disease of the first few months of infancy. It is caused by anti-SSA and anti-SSB antibodies, which are products of maternal autoimmune disorders (SLE, Sjögren, rheumatoid arthritis) and can be passively transported across the placenta. The prevalence of NLE is low. The major clinical findings are cutaneous (typical annular erythematous plaques), cardiac, hepatic and hematologic alterations. Its most severe consequence is third-degree heart block, which is irreversible, requires pacemaker-implantation and responsible for the 20-30% mortality rate. Symptoms usually resolve spontaneously at age of 6-9 months in association with disappearance of maternal antibodies from the infant's serum. In our case the typical cutaneous manifestations covered virtually the whole body, were present at birth, however, no conduction defects developed. The fact that the mother's sickness was not known at birth made it difficult to establish the diagnosis. The significant thrombocytopaenia, progressive skin-changes and the elevated liver function tests necessitated systemic steroid treatment.
Vass
Géza
G
Semmelweis Egyetem, Altalános Orvostudományi Kar, I. Gyermekgyógyászati Klinika, Budapest, Budenz u. 18. 1021. vassgeza@yahoo.com
Antal
Zsuzsanna
Z
Katona
Mária
M
Kárpáti
Sarolta
S
Balogh
Lídia
L
D001323:Autoantibodies / Q000097:blood; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007231:Infant, Newborn; D008111:Liver Function Tests; D008178:Lupus Erythematosus, Cutaneous / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology; D008180:Lupus Erythematosus, Systemic / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology; D011239:Prednisolone / Q000627:therapeutic use; D013921:Thrombocytopenia / Q000209:etiology
D001323:Autoantibodies; D005938:Glucocorticoids; D011239:Prednisolone