Why Neonatal Lupus Erythematosus Should Be Followed Over Longer Term

Cutaneous neonatal lupus erythematosus can exhibit long-term cutaneous residua. Findings underlie the importance of accurate diagnosis, long-term monitoring, and appropriate counseling.

Findings from a study published in the Journal of the American Academy of Dermatology suggest that despite resolution of symptoms without treatment, residual cutaneous changes from neonatal lupus erythematosus may be persistent and contribute to future skin problems in childhood, particularly scarring.

The retrospective study included 106 infants treated for cutaneous changes from neonatal lupus erythematosus at a specialized neonatal lupus erythematosus rheumatology clinic at the Hospital for Sick Children in Toronto, Ontario, Canada, between 1980 and 2017. Mean onset of the first signs of a rash were at 5.5 weeks of age, and mean age at diagnosis was 11.6 weeks.

In the study, rash resolved completely in 66% of patients (70/106) at a mean age of 5.7 months. The remaining 34% (36 patients) had persistent residual skin manifestations at the time of the final follow-up visit (mean age, 3.1 years), including persistent telangiectasia in 13%, hypo- or hyperpigmentation in 17%, and atrophic scarring in 9%. Systemic signs were also present in many of the infants, including hepatitis, hematologic signs, congenital heart block, and hydrocephalus in 43%, 33%, 9%, and 9% of patients, respectively.

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Two-thirds of the cohort (69/106 patients, 65%) had documented signs of maternal connective tissue disease, most of which (71%) was confirmed to be systemic lupus erythematosus. Of the mothers, 94% carried Anti-Ro antibodies, 69% were anti-La positive, and 13% had anti-U1 RNP antibodies, either with or without anti-Ro or anti-La.

Limited duration of follow-up in some patients and the retrospective nature of the study were cited by the researchers as limitations. Selection bias and the small sample size were also mentioned.

The study found a significant correlation between the presence of skin lesions at the time of birth and later atrophic scarring, which might be attributed to an inflammatory insult in utero, the investigators suggested. They determined that although cutaneous changes from neonatal lupus erythematosus is a rare condition, the potential for future sequelae makes it necessary to accurately diagnose and effectively counsel parents about the condition, and to continue to monitor children who developed signs shortly after birth for future cutaneous sequelae.

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Levy R, Briggs L, Silverman E, Pope E, Lara-Corrales I. Cutaneous sequelae in neonatal lupus: A retrospective cohort study [published online October 15, 2019]. J Am Acad Dermatol. doi:10.1016/j.jaad.2019.09.083