Neonatal lupus erythematosus is characterized by annular or plaque-type erythemato-squamous skin lesions usually of the face and scalp that develop in 2–10 % of children of anti-SSA/Ro- or anti-SSB/La-positive mothers at birth or, more often, within the first weeks of life. Some children have periorbital erythemas. The skin lesions usually resolve spontaneously within weeks to months; the persistence of telangiectasia or postinflammatory hyperpigmentations is rare. In one third of the children with skin symptoms, other organ systems are involved with the risk of an atrioventricular block, elevated liver enzymes, hyperbilirubinemia, and decreased numbers of peripheral lymphocytes or thrombocytes. The most threatening manifestation is a grade III AV block which may require pacemaker placement. In known anti-Ro antibody-positive mothers, the respective diagnosis and, if needed, appropriate therapy can be performed in utero, and a preventive therapy of other manifestations of neonatal lupus with corticosteroids may be considered.
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