Neonatal rash is much more than skin deep

IMAGE CREDIT / AUTHOR SUPPLIED

THE CASE

The frightened mother of a vigorous, healthy 14-day-old girl brings her daughter to you for an urgent consultation regarding a facial rash that has blossomed since a few subtle spots were noted at birth. What’s your diagnosis?

DIAGNOSIS:

Neonatal lupus erythematosus

Epidemiology and pathogenesis

Neonatal lupus erythematosus (NLE) is an uncommon autoimmune process caused by transplacental passage of maternal antibodies. It occurs in 1 of every 20,000 live births in the United States, with female neonates more predominantly affected than males (3:1 ratio).¹ It occurs in 1% to 2% of babies born to mothers with autoimmune disease² who possess anti-SSA/Ro, anti-SS/La, and/or anti-U1-ribonucleoprotein (U1-RNP) antibodies. Anti-SSA/Ro is positive in more than 90% of cases.³

Clinical presentation

Clinical NLE typically presents with dermatologic and/or cardiac symptoms.

Two-thirds of children with dermatologic manifestations have lesions present at birth, and the remainder develop lesions within 2 to 3 months postnatally.⁴  The cutaneous eruption develops as annular erythematous plaques or arcuate macules with a slight scale and raised red borders. Atrophy, dyspigmentation, and/or telangiectasias may be present. The rash is photosensitive, may spread dramatically after sun exposure, and is most often located on the face and scalp.⁵

The most common and serious manifestation of NLE is congenital heart block. First detected by fetal ultrasound between 20 and 24 weeks’ gestation, NLE is responsible for 85% of all cases of congenital heart block.³ The incidence of this complication is 1% in mothers positive for anti-SSA/Ro antibodies, but rises to 25% in mothers with these antibodies who have had a previous child with congenital heart block.

Neonatal lupus often leads to transient asymptomatically elevated liver enzymes, which can rarely lead to hepatitis and liver failure. Its most common hematologic manifestation is thrombocytopenia, which may result in clinically apparent petechiae.⁶

Differential diagnosis

The differential diagnosis of isolated polycyclic skin lesions in a neonate includes seborrheic dermatitis, tinea corporis, urticaria, and erythema marginatum. The differential diagnosis of isolated annular erythematous lesions includes erythema multiforme, erythema annulare centrifugum, and Pityrosporum dermal infection. However, NLE should be considered in any newborn with an annular skin eruption.

Prevention and treatment

Some investigators recommend the use of hydroxychloroquine prophylactically beginning at 6 to 10 weeks’ gestation in women who have previously given birth to a child with NLE and cardiac block.⁷ However, data is preliminary, and further studies are needed.

The cutaneous rash is self-resolving as maternal antibodies leave the neonatal circulation, with a mean time to resolution of 4 months.⁸ It is important to encourage sun protection because the lesions are photosensitive. Some investigators recommend low-potency topical steroids for 2 to 4 weeks.⁹ Most cases are nonscarring, although dyspigmentation may persist in darkly pigmented infants for months to years, and telangiectasias may persist indefinitely.^9,10 Fetuses with second-degree heart block may be treated in utero by maternal administration of glucocorticoids, while children with third-degree heart block will likely need pacemaker implantation.^6,11

Prognosis

Neonatal lupus erythematosus with cardiac involvement is associated with a 20% to 30% mortality in the neonatal period, with children with congenital heart block and concurrent cardiomyopathy experiencing the highest mortality.⁶ Fortunately, NLE’s dermatologic, hepatic, and hematologic manifestations are rarely associated with permanent sequelae.⁶

Although 50% of mothers are asymptomatic at delivery, they are at risk for developing autoimmune disorders during the subsequent decade, including systemic lupus erythematosus and Sjögren syndrome.¹²

REFERENCES

1. Emer J, Luber A, Yazdani M, Marciniak B, Sidhu H, Phelps R. Neonate with annular plaques of the scalp. J Clin Aesthet Dermatol. 2013;6(6): 43-47.

2. Al-Osaimi H, Yelamanchili S. Neonatal lupus erythematosus (NLE). In: Almoallim H, ed. Systemic lupus erythematosus. Available from: http://www.intechopen.com/books/systemic-lupus-erythematosus/neonatal-lupus-erythematosus. Published March 21, 2012. Accessed November 11, 2013.

3. Crowley E, Frieden IJ. Neonatal lupus erythematosus: an unusual congenital presentation with cutaneous atrophy, erosions, alopecia, and pancytopenia. Pediatr Dermatol. 1998;15(l):38-42.

4. Elish D, Silverberg NB. Neonatal lupus erythematosus. Cutis. 2006;77(2):82-86.

5. 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.

6. Hon KL and Leung AK. Neonatal lupus erythematosus. Autoimmune Dis. 2012 (2012), p.301274 (6 pages).

7. Izmirly PM, Costedoat-Chalumeau N, Pisoni CN, et al. Maternal use of hydroxychloroquine is associated with a reduced risk of recurrent anti-SSA/Ro-antibody-associated cardiac manifestations of neonatal lupus. Circulation. 2012;126(1):76-82.

8 Neiman AR, Lee LA, Weston WL, Buyon JP. Cutaneous manifestations of neonatal lupus without heart block: characteristics of mothers and children enrolled in a national registry. J Pediatr. 2000;137(5):674-680.

9. McKinlay JR, Cooke LM, Cunningham BB, Gibbs NF. Neonatal lupus erythematosus. J Am Board Fam Pract. 2001;14(1):68-70.

10. High WA, Costner MI. Persistent scarring, atrophy, and dyspigmentation in a preteen girl with neonatal lupus erythematosus. J Am Acad Dermatol. 2003;48(4):626-628.

11. Saleeb S, Copel J, Friedman D, Buyon JP. Comparison of treatment with fluorinated glucocorticoids to the natural history of autoantibody-associated congenital heart block: retrospective review of the research registry for neonatal lupus. Arthritis Rheum. 1999;42(11):2335-2345. 

12. Rivera TL, Izmirly PM, Birnbaum BK, et al. Disease progression in mothers of children enrolled in the Research Registry for Neonatal Lupus. Ann Rheum Dis. 2009;68(6):828-835.

MS PASCOE is a fourth-year medical student at Johns Hopkins University School of Medicine, Baltimore, Maryland. DR COHEN, the section editor for Dermatology: What’s Your DX?, is director, Pediatric Dermatology and Cutaneous Laser Center, and associate professor of pediatrics and dermatology, Johns Hopkins University School of Medicine, Baltimore. The author and section editor have nothing to disclose regarding affiliations with or financial interests in any organizations that may have an interest in any part of this article. Vignettes are based on real cases that have been modified to allow the author and editor to focus on key teaching points. Images also may be edited or substituted for teaching purposes.