Diagnosis: Neonatal lupus erythematosus

The baby was diagnosed with neonatal lupus erythematosus (NLE). A skin biopsy taken from her back revealed interface dermatitis with dyskeratosis and increased dermal mucin. Echocardiogram and ECG were unremarkable. Complete blood count, liver function tests, and renal function were normal. Our patient and her mother had similar antibody profiles; both were positive for anti-Ro antibody and to a lesser
degree antinuclear antibody. 

A rare disease, NLE is caused by transplacental passage of maternal anti-Ro antibodies and occurs in 1% of neonates born to anti-Ro-positive mothers. A mother who has one child with NLE has a 25% chance of having another child who is also afflicted. (Our patient had no similarly affected siblings.)

Patients typically present with an annular, erythematous, scaly rash before the age of three months. The rash is most often located on the face in a periorbital distribution. A report of 18 NLE patients noted lesion distribution as follows: face (17), periorbital skin (14), scalp (15), arms and legs (13), and trunk and groin (13).1 The skin lesions generally resolve on their own within a year. Usually, there is no scarring, but some patients exhibit mild atrophy and/or telangiectasias.

The most common extracutaneous manifestations of NLE are cardiac, hepatic, and, less commonly, hematologic abnormalities. Congenital heart block is seen in 15%-30% of infants with NLE. Generally, the heart block is present at birth, but in rare cases, it can develop after birth; therefore, careful monitoring is important. If the heart is affected, the mortality is 20%, usually during the first few years of life, and two thirds of children require pacemakers.2

Hepatic and hematologic disease may be present at birth but can also manifest in the first few months of life. The three presentations of hepatic disease in NLE patients include severe liver failure during gestation or in the neonatal period, conjugated hyperbilirubinemia, and mild elevations of aminotransferases at two to three months of age.

The primary treatment for skin lesions is mild topical steroids. Photoprotection is also strongly encouraged. Skin lesions, hepatic disease, and hematologic disease resolve with clearance of maternal antibodies. NLE patients generally do not have an increased risk of future autoimmune disease.

Our patient was discharged and had subsequent improvement of her facial rash. Then at two months of age, she was readmitted with fever, a new rash extending onto her trunk and extremities, and mild elevations of her aminotransferases. Infectious causes for her fever and her aminotransferase elevations were ruled out. Biopsy of the new rash showed lupus erythematosus. She began to improve with administration of systemic corticosteroids. 

Our patient’s mother had not been diagnosed with an autoimmune disease. We strongly encouraged her to see her personal clinician. Mothers of NLE patients either have or eventually develop a defined autoimmune disorder.

Dr. Yang is a resident in dermatology at Baylor College of Medicine in Houston.

 

References

1. Weston WL, Morelli JG, Lee LA. The clinical spectrum of anti-Ro-positive cutaneous neonatal lupus erythematosus. J Am Acad Dermatol. 1999;40(5 Pt 1):675-681.
2. Buyon JP, Hiebert R, Copel J, et al. Autoimmune-associated congenital heart block: demographics, mortality, morbidity and recurrence rates obtained from a national neonatal lupus registry. J Am Coll Cardiol. 1998;31:1658-1666.