Neonatal lupus erythematosus.

The patient is a newborn girl, born at term by cesarian section, weighing 3,550 g, and measuring 50 cm in length. According to her mother, she had been presenting erythematous spots involving the scalp, face, and trunk since birth that had increased in size with time. The mother denied any other changes and reported normal growth and development. Physical examination at 2 months of age revealed an infant in good general condition, hydrated, with no fever and no abnormalities detected on careful physical examination. The dermatologic examination revealed numerous circumscribed erythematous-brownish flat maculae with sharp borders and irregular contours, with no follicular hyperkeratosis, but with telangiectasias and areas of atrophy. They were lenticular and nummular, especially on the face (peri-orbital heliotropic lesions), and appeared as plaques on the trunk, with a tendency to confluence. The lesions also involved the scalp, neck, and extremities (Fig. 1). Histopathologic examination revealed an atrophic epidermis with focal areas of hydropic degeneration of the basal layer. A discrete perivascular and periannexal lymphohistiocytic infiltrate was observed in the superficial dermis, with melaninophages, frequent extravasation of red blood cells, angiectasia, and edema (Fig. 2). Serologic tests were reactive for antinuclear factor (ANF) (titer 1:100) of the speckled pattern, the presence of anti-Ro antibodies, and absence of anti-RNA and anti-Sm antibodies (Table 1). Blood counts and electrocardiogram were normal (Table 1). HLA typing showed positivity for DR-3 (Table 2). At 5 months of age the patient already showed a marked improvement of the skin lesions with only some areas of discrete pigmentation, a few atrophic areas, and rare telangiectasis (Fig. 3). The serologic tests (ANF, anti-Ro) had become nonreactive and the anti-RNA and anti-Sm tests continued to be negative. Examination of the mother revealed an asymptomatic 25-year-old woman reporting no manifestations suggestive of lupus. General and special physical examination revealed no abnormalities. Dermatologic examination showed no active or residual lesions of discoid or systemic lupus erythematosus. The pregnancy had been uneventful. Histopathologic examination of the girl's skin revealed the epidermis without obvious changes, minimal edema in the dermis, and a discrete perivascular inflammatory mononuclear cell infiltrate. Direct immunofluorescence of normal skin not exposed to the sun was negative. Blood counts revealed mild anemia and a tendency to leukopenia and thrombocytopenia (Table 1). Serologic tests showed reactive ANF (titers 1:400 and 1:800, speckled pattern), the presence of anti-Ro antibodies, and the absense of anti-RNA and anti-Sm antibodies (Table 1). HLA-typing revealed positivity for DR-3 (Table 2).