Case

A full-term, healthy boy of African American ancestry was delivered via spontaneous vaginal delivery without any complications. The 36-year-old mother had a significant medical history of gestational diabetes and systemic lupus erythematosus. The patient’s initial hospital course after delivery was significant for hyperbilirubinemia, and he received inpatient treatment via fluorescent white light phototherapy. He received all appropriate vaccinations and was discharged. Follow-up outpatient appointments demonstrated an appropriately developing newborn, with normal weight gain. However, at his 1-month visit, the patient was noted to have annular, red plaques to his scalp and face (Figure). His vital signs were within normal limits for his age. A full systemic evaluation was performed, with no additional findings.

Evaluation and testing

Patient labs at the time of presentation were notable for positive anti–Ro/SS-A antibodies. Complete blood count and comprehensive metabolic panel results were within normal limits. Maternal serology was also found to be positive for anti–Ro/SS-A antibodies. A punch biopsy of a forehead lesion was performed to confirm the diagnosis.

Diagnosis

Neonatal lupus erythematosus (NLE)

Differential diagnosis

The differential diagnosis of NLE includes conditions such as tinea corporis, annular urticaria, and seborrheic dermatitis, as well as rarer conditions such as erythema annulare centrifugum and annular erythema of infancy. An extensive, while not exhaustive, list of differential diagnoses is explored in the Table.^1-9

Discussion

NLE is an autoimmune disease that occurs due to the transplacental transfer of anti–Ro/SS-A or La/SS-B antibodies from mother to fetus.¹ It is estimated that 10% of babies born to mothers with positive anti–Ro/SS-A or anti–La/SS-B antibodies will develop 1 or more features of NLE.² Like systemic lupus erythematosus, NLE can involve multiple organs; however, the skin and heart are predominantly affected.³ One percent to 2% of infants born to mothers with positive antibodies can have congenital heart block (CHB); this risk increases to 17% if the mother has had a previous child with CHB.² Neonatal lupus is estimated to be responsible for 85% of cases of CHB.¹

Cutaneous manifestations of NLE include annular erythematous or polycystic plaques, with or without scale, most commonly on the neck, face, or scalp.¹ Periorbital erythema and discoid, bullous, or atrophic lesions have also been noted.^1,3 Cutaneous lesions may be present at birth or appear in the first few weeks of life. They are typically benign and self-limited, resolving within weeks to months, but can result in residual scarring. Diagnosis is made clinically with positive anti–Ro/SS-A or La/SS-B serology, although punch biopsy may be used to confirm the diagnosis and rule out other conditions. Treatment of cutaneous NLE is primarily supportive in nature. Observation and photoprotection are the mainstay of treatment; topical steroids are routinely unnecessary.¹

NLE should always be on the differential diagnosis of annular lesions in an infant, especially if the mother has a known history of autoimmune disease or has previously tested positive for anti–Ro/SS-A or La/SS-B autoantibodies. Infants with NLE or born to mothers with anti–Ro/SS-A or anti–La/SS-B antibodies should have an echocardiogram to rule out CHB, with prompt referral to a cardiologist in case of any abnormalities.¹ If maternal positive antibody status is known before delivery or she has had a previously affected child, serial fetal echocardiography is recommended as a screening tool starting at 16 weeks’ gestation.²

Patient course

An electrocardiogram was performed to screen for heart block, which was unremarkable. The child’s parents were educated on the self-resolving, benign nature of the condition. Photoprotection with loose, protective clothing and sun protection factor was encouraged. By the time the patient reached 6 months of age, the lesions had resolved.

References:

1. Hon KL, Leung AK. Neonatal lupus erythematosus. Autoimmune Dis. Published online September 2, 2012. doi:10.1155/2012/301274
2. Dao KH, Bermas BL. Systemic lupus erythematosus management in pregnancy. Int J Womens Health. 2022;14:199-211. doi:10.2147/ijwh.s282604
3. Saoji V, Deopujari S. Neonatal lupus erythematosus-three different presentations. Indian Journal of Paediatric Dermatology. 2014;15(3):110-113. doi:10.4103/2319-7250.143658
4. Sahoo AK, Mahajan R. Management of tinea corporis, tinea cruris, and tinea pedis: a comprehensive review. Indian Dermatol Online J. 2016;7(2):77-86. doi:10.4103/2229-5178.178099
5. Örnek S, Zuberbier T, Kocatürk E. Annular urticarial lesions. Clin Dermatol. 2022;40(5):480-504. doi:10.1016/j.clindermatol.2021.12.010
6. Dall’Oglio F, Nasca MR, Gerbino C, Micali G. An overview of the diagnosis and management of seborrheic dermatitis. Clin Cosmet Investig Dermatol. 2022;15:1537-1548. doi:10.2147/ccid.s284671
7. Trayes KP, Love G, Studdiford JS. Erythema multiforme: recognition and management. Am Fam Physician. 2019;100(2):82-88.
8. Carlesimo M, Mari E, Abruzzese C, et al. What is erythema annulare centrifugum? a familial case. Eur J Inflamm. 2013;11(2):531-533. doi:10.1177/1721727x1301100223
9. Ly S, Guram M, Zoumberos N, Kincannon J, Evans MS. Annular erythema of infancy: a rare and challenging diagnosis. Pediatr Dermatol. 2023;40(4):681-687. doi:10.1111/pde.15238