Setting.
—The pediatric department in a tertiary-care hospital.

Interventions.
—Packed red blood cell transfusions and a 3-week course of high-dose steroid therapy.

Measurements/Main Results.
—The patient presented with severe anemia and a circumscribed, reticular, macular rash on the face and neck at 5 months of age. Skin lesion biopsy revealed epidermic hyperkeratosis, hydropic degeneration of the basal layer, and deposition of immunoglobulins and granular C1 q at the dermoepidermal junction. Ro/SS-A antibodies were present in the infant. BFU-E (erythyroid progenitor burst-forming unit) colonies in bone marrow increased by about tenfold when suppressor CD8⁺ T lymphocytes were removed, indicating immune suppression of hematopoiesis. High-dose steroid therapy failed. The infant subsequently developed gram-negative sepsis, severe metabolic acidosis, and consumptive coagulopathy and died.

Conclusions.
—Neonatal lupus erythematosus may present as part of a spectrum. The disease may range from mild and transient to a severe, life-threatening condition requiring immediate intervention, as in the case reported here. This is the first report of neonatal lupus associated with aplastic anemia due to immune-mediated suppression of hematopoiesis.

(AJDC. 1993;147:941-944)