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A 2-month-old develops a rash after a sunny day in the park
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A 2-month-old male presents with a 3-week history of rash initially on his forehead and later noted on his groin, right foot and trunk. The patient had a trial of antifungal cream with no improvement. He is otherwise in good health with no significant medical or surgical history. The rash initially appeared 3 days after sun exposure; more specifically, the mother remembered that she changed the patient’s diaper during an outdoor family picnic on a recent sunny day.
Shehla Admani, MD
On physical exam, the patient is noted to have numerous pink, round, somewhat targetoid papules and plaques with central hemorrhagic crust on his face (Figure 1). He has a few pink, round papules and plaques on his abdomen, right inguinal region, left lower extremity and penis (Figure 2). He is also noted to have a larger, round, eroded plaque with central hemorrhagic crust on the sole of his right foot (Figure 3).
Further work-up of this patient could reveal which of the following:
- A. Heart block
- B. Transaminitis
- C. Thrombocytopenia
- D. Leukocytosis
- E. All of the above
Treatment of these potentially scarring skin lesions should consist of:
- A. Topical antibiotic
- B. Topical antifungal
- C. Sunscreen
- D. Topical corticosteroid
- E. C and D
Diagnosis: Neonatal lupus erythematosus
Neonatal lupus erythematosus (NLE) is an autoimmune disease caused by transplacental passage of maternal autoantibodies against Ro/SS-A, La/SS-B and, less commonly, U1-ribonucleoprotein (U1-RNP). It is a rare condition and estimated to occur in one in 20,000 live births. NLE is seen in less than 2.5% of infants born to mothers with these autoantibodies, and a great majority of mothers are asymptomatic at the time of delivery. The clinical spectrum of NLE consists of cutaneous, cardiac and systemic abnormalities. The course is generally benign and self-limited; however, NLE may be associated with serious sequelae.
Figure 1 shows a face with numerous pink, round, somewhat-targetoid papules and plaques with central hemorrhagic crust.
Images: Krakowski AC
The most common clinical manifestation of NLE is a cutaneous eruption consisting of annular erythematous plaques with or without fine scale that can be present at birth or appear in the first few weeks of life. The rash can appear after sun exposure and is similar to that seen in subacute lupus erythematosus, rather than the “classic” malar rash. The rash of NLE generally resolves by 6 months of age, at around the same time the maternal autoantibodies are cleared from the infant’s circulation. Treatment consists of avoidance of sun exposure, sunscreen and topical corticosteroids.
Figures 2 and 3. Figure 2 is a round, erythematous papule on shaft of penis. Figure 3 shows the patient’s sole of the right foot has a 3 cm erythematous round partially eroded plaque with central hemorrhagic crust.
The second most common manifestation is cardiac and can consist of conduction abnormalities and/or cardiomyopathy. Cardiac involvement has a 15% to 30% mortality rate and requires regular monitoring of cardiac function and, in some cases, a pacemaker. Hepatobiliary and hematologic manifestations include elevated liver enzymes, conjugated hyperbilirubinemia, mild hepatomegaly, neutropenia and thrombocytopenia. These are usually self-limited; however, severe cases may require systemic corticosteroids, IVIG and/or immunosuppressive agents.
The patient in our case had further work-up showing positive SS-A; negative SS-B; negative RNP; and elevated aspartate aminotransferase, alanine aminotransferase and alkaline phosphatase. Complete blood count and electrocardiogram were within normal limits. The patient’s mother had a history of systemic lupus erythematosus and rheumatoid arthritis. It is interesting to note that our patient had his diaper changed in the sun leading to the development of a lesion in the diaper area (a location that is not typical of this condition), as seen in Figure 3. He was treated aggressively with sun protection and 2 weeks of fluocinonide ointment twice daily, leading to near complete-resolution of the skin lesions.
References:
Buyon JP. Lupus. 2004;13:705-712.
For more information:
Shehla Admani, MD, is a Clinical Research Fellow in Pediatric Dermatology at Rady Children’s Hospital, San Diego. She can be reached at 8010 Frost St., Suite 602, San Diego, CA 92123; email: sadmani@rchsd.org.
Andrew C. Krakowski, MD, is an attending physician at Rady Children’s Hospital, San Diego.