Affiliation:
1. UOC di Neonatologia e Terapia Intensiva Neonatale, AO Osp. Riuniti “Villa Sofia-Cervello”, Palermo
2. Dipartimento PROMISE, Università di Palermo
Abstract
Neonatal lupus erythematosus (NLE) is a rare immune-mediated disorder due to the pas- sage of maternal anti-Ro/SSA and anti-La/SSB antibodies through the placenta to the foe- tus and newborn. Major manifestations include cardiac anomalies and skin lesions, while hepatic and haematological abnormalities are observed in a minority of cases. The most serious complication is congenital heart block, usually already occurring in the prenatal period (second or third trimester of pregnancy). Cutaneous lupus is the typical manifestation of NLE, characterized by transient and generally photosensitive annular erythematous le- sions, preferentially localized on the face, scalp or periocular region, and less commonly observed on the trunk and extremities. Due to its benign and transient course, no treatment is required. Prophylaxis with hydroxychloroquine in pregnant women testing positive for autoantibodies and/or with a previously affected child may reduce the risk of cardiac and/or cutaneous complications in newborns and infants. The article reports the case of a two-day-old newborn showing a widespread cutaneous rash with erythematous “stepped” patches compatible with annular erythema, localized on their upper and lower limbs, as well as on the anterior surface of their trunk. Obstetric history, which revealed the mother being affected with Sjogren Syndrome and performing hydroxychloroquine prophylaxis, led to the NLE diagnostic suspicion, later confirmed by the detection of specific antibodies (anti-SSA/Ro60 and SSA/Ro52). Although the prognosis of cutaneous NLE is generally favourable, any coexisting and potential life-threatening complications, including haema- tological and cardiological abnormalities, should be ruled out.