NEONATAL LUPUS ERYTHEMATOSUS

By Gary M. White, MD

Neonatal lupus erythematosus


Neonatal lupus erythematosus (NLE) is a disease of neonates born to mothers positive for SS-A (Ro) or SS-B (La) antibodies.

Epidemiology

A high preponderance of female infants are affected. Both the mother and the infant may be found to have SS-A (Ro), SS-B (La) antibodies or rarely U1RNP. Mothers may have symptoms of Sjögren's syndrome or lupus erythematosus at the time of delivery or they may go on to develop them later.

Clinical

Annular, erythematous lesions often with scale and atrophy on the scalp or face in the first two months of life are characteristic of neonatal lupus erythematosus. Prominent telangiectatic macules and angiomatous papules may be present early and occur unrelated to an inflammatory component. The angiomatous papules are particularly common in the inguinal or vulvar area. Depressed lesions of lupus profundus may form and can be permanent [BJD 1997;136;112].

Systemic

Congenital heart block may be associated and is correlated with mortality. Less common associations include thrombocytopenia with petechiae, hepatitis, hemolytic anemia, hydrocephalus (e.g., manifested by enlarging head circumference at 1-3 months of age) and aplastic anemia.

Annular Erythema of Infancy (AEI)

AEI is a somewhat poorly defined condition in which annular lesions occur in the newborn. It has been thought to be a hypersensitivity response to antigens present in the newborn period. Some of these patients have had mothers with lupus, so there may be an overlap in some cases between AEI and neonatal lupus.

Treatment

Skin lesions should be managed with sunscreen and sun avoidance. They usually resolve within 6 months on their own along with the disappearance of the antibody. Mild topical steroids for a short period of time may be tried. A CBC and LFTs should be checked. Cardiac status should be assessed by a non-dermatologist. A pacemaker may be needed.

Additional Pictures

Neonatal lupus erythematosus

Neonatal lupus erythematosus

Neonatal lupus erythematosus

Courtesy of Theodore Sebastian, MD
Neonatal lupus erythematosus

References

Indian Dermatology Online Journal

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