Cutaneous Neonatal Lupus Erythematosus

The skin lesions of NLE represent subacute cutaneous LE (SCLE), although with a distribution distinct from that of SCLE in adults. Cutaneous NLE is characterized by annular, erythematous, nonscarring, photosensitive plaques, sometimes associated with hypopigmentation (Weston et al. 1999). Occasionally, lesions reminiscent of cutis marmorata congenita occur. The onset is generally in the first few weeks of life, although lesions have been noted at birth in several cases. There is a predilection for the head, in particular the periorbital skin, but lesions may occur at any site. The affected periorbital skin has been described as having an "owl eye," "eye mask," or "raccoon eye" appearance. Disease activity resolves in a few weeks or months, but dyspigmentation may persist for several months, and in some cases there are residual telangiectasias (Thornton et al. 1995).

The risk for a child with cutaneous NLE to have extracutaneous features is not precisely known. It has been estimated that approximately 10% of children with cutaneous NLE also have cardiac disease (Lee 1993). An examination of records from a national NLE registry indicates that 23% of children with cutaneous NLE without cardiac disease have hepatobiliary or hematologic findings (Neimann et al. 2000). Thus, perhaps one third of the cases of cutaneous NLE have extracutaneous manifestations.

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