lupus

A Neonate With Skin Lesions: What’s the Cause?

Paul Redmond, MD; Benjamin Dillard, MD; and Melissa Nan Frascogna, MD

A 4-week-old girl presented to the pediatric emergency department (PED) with a rash. The patient’s mother reported that over the last 5 days she had noted what appeared to be “insect bites” on her infant, first appearing over the right eyebrow and spreading to include the forehead, ears, scalp, back, and sparsely over the extremities. The lesions began as small erythematous papules that subsequently widened, developing a pale halo below erythematous borders and dusky central clearings. 

The rash did not appear to bother the newborn. There had been no pustules or vesicles, and she continued to eat well, have normal bladder and bowel function, and remain afebrile. No associated seizures nor maternal herpes infections were reported. Birth history was notable for maternal chorioamnionitis and subsequent concern for neonatal sepsis, resulting in a brief neonatal intensive care unit (NICU) stay. Perinatal brain imaging results were unremarkable, and echocardiogram results were notable for patent foramen ovale and mild right ventricular hypertension. She was discharged home after 3 days in the NICU and had been in normal health until the appearance of the rash.

neonatal lupus

What is the cause of this rash?

(Answer and discussion on next page)

Answer: Neonatal lupus erythematosus

The initial differential diagnosis included erythema multiforme precipitated by a herpes simplex virus infection, other infection, or medication exposure. Because of a questionable apneic spell while in the PED, a sepsis evaluation was performed, and the patient was admitted. Broad-spectrum antibiotics and acyclovir were started empirically with culture results pending. Results of laboratory evaluations, including routine cerebrospinal fluid studies and HSV polymerase chain reaction all were unremarkable. A dermatologist was consulted, and further laboratory evaluations revealed positive serum antinuclear antibody, anti-Smith, anti-Ro, and anti-La antibodies confirming the diagnosis of neonatal lupus erythematosus (NLE). The patient was seen for follow-up in the pediatric rheumatology and dermatology clinics with improving symptoms.

Rashes presenting in the newborn period are commonly benign; however, they may also be the sign of underlying systemic disease.1

The rash of NLE is well-demarcated, erythematous, annular patches most commonly on the scalp, face, and neck. It typically appears days to weeks after birth, often after sun exposure.2 NLE may occur without a noted history of maternal lupus, heart block, liver involvement, or thrombocytopenia; 50% of mothers will be asymptomatic.3 It can present in any neonate born to mothers with autoimmune disease and is mediated by passive transmission of maternal anti-Ro and anti-La antibodies. 

Although not every mother will manifest clinical features of lupus or Sjögren syndrome, NLE should be considered in any newborn presenting with an annular rash. Skin findings normally resolve within the first 6 months of life, and therapy is focused on moisturizers and sun protection. Infants should be followed by a rheumatologist, as there is evidence that affected children have an increased risk of developing autoimmune disorders. However, the risk of systemic lupus erythematous does not appear to be elevated.4 n

Paul Redmond, MD, is a resident in the department of pediatrics at the University of Mississippi Medical Center in Jackson.

Benjamin Dillard, MD, is a professor in the department of pediatrics, division of emergency medicine, at the University of Mississippi Medical Center in Jackson.

Melissa Nan Frascogna, MD, is an associate professor in the department of pediatrics, division of emergency medicine, at the University of Mississippi Medical Center in Jackson.

References

1. Su J. Common rashes in neonates. Aust Fam Physician. 2012;41(5):280-286.

2. Warner AM, Frey KA, Connolly S. Annular rash on a newborn. J Fam Pract. 2006;55(2):127-129.

3. Barcelos A, Fernandes B. Cutaneous manifestations of neonatal lupus: a case report. Acta Reumatol Port. 2012;37(4):352-354.

4. Inzinger M, Salmhofer W, Binder B. Neonatal lupus erythematosus and its clinical variability. J Dtsch Dermatol Ges. 2012;10(6):407-411.