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| Fig. 22.4 Neonatal lupus erythematosus demonstrating sharply defined erythema of the scalp and face. Periocular involvement producing a 'raccoon eyes' appearance is common and is strongly suggestive of the diagnosis. (Courtesy of the Fitzsimons Army Medical Center teaching files.) |
In NLE, infants develop skin disease (50%), heart disease (50%), or both (10%). The skin lesions occur most commonly on the face and head (Fig. 22-4), morphologically resemble SCLE lesions, and are transient, resolving within a few months, but sometimes leaving atrophic lesions. The heart disease usually manifests as isolated complete heart block, although lesser degrees of heart block have been reported. The heart block is generally permanent and may require a pacemaker. About 10% of infants with NLE and heart disease die from cardiac complications. A few infants with NLE also have thrombocytopenia and and/or liver disease.
Nearly all infants with NLE have anti–Ro/SS-A and sometimes anti–La/SS-B antibodies, as will their mothers. A few NLE patients have been reported to have anti-U1RNP antibodies in the absence of anti-Ro or anti-La antibodies.These antibodies are transient and are not detectable after a few months of life. They are of maternal origin, transferred via the placenta.
Lee LA: Neonatal lupus erythematosus,
J Invest Dermatol 100:9S–13S, 1993.
