Maternal and fetal outcome in neonatal lupus erythematosus.

Maternal health, children's health, and obstetric histories were assessed in a follow-up study of 21 families with children with neonatal lupus erythematosus; this group constituted approximately 15% of all reported cases. Twenty-one mothers had twenty-four children with the disease. Twelve children had congenital heart block (5 boys, 7 girls), 10 had cutaneous lupus lesions (1 boy, 9 girls), and 2 girls had both heart block and cutaneous lesions. Although half of the mothers were initially asymptomatic, 18 of 21 have developed symptoms during the follow-up period (range, 0.25 to 9.5 years). Three of the children died in the neonatal period. The 21 children who survived have been asymptomatic during follow-up, although 5 of 11 with heart block have pacemakers. The mothers did not have an increased risk for spontaneous abortions. Three of twelve livebirths after the birth of the first child with neonatal lupus erythematosus resulted in another affected child.

Systemic lupus erythematosus in a boy with chronic granulomatous disease: case report and review of the literature.

We describe a patient with X-linked chronic granulomatous disease (CGD) who developed systemic lupus erythematosus, which was characterized by photosensitivity, malar rash, glomerulonephritis, leukopenia, hypocomplementemia, antinuclear antibodies, and anti-double-stranded DNA antibodies, at age 3. The patient's mother is an asymptomatic carrier of CGD, and her other son (the patient's half-brother) also has CGD. Neither the mother nor the brother has clinical or serologic evidence of systemic lupus erythematosus. Previous cases of discoid lupus-like skin lesions have been reported both in carriers and in patients with CGD. Our patient represents the first reported case of an individual with convincing clinical, serologic, and pathologic evidence of systemic lupus erythematosus. The association between defective host defense mechanisms and autoimmune phenomena has been described previously in patients with Job's syndrome and in patients with B cell and T cell deficiency disorders, including the acquired immunodeficiency syndrome. The relationship between the known leukocyte defects in CGD and the pathogenesis of a lupus-like illness is unclear.