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Published on: September 20, 2018
Periorbital cutaneous neonatal lupus
Rani M Cooper1, David F Butler, Fred Ghali
1Department of Medicine, Division of Dermatology, Scott and White Memorial Hospital and Clinic, 409 Adams Street, Temple, TX 76508, USA.
Insights
This study highlights a rare facial rash in infants, often linked to maternal autoimmune conditions like Sjögren syndrome. Early diagnosis and treatment, including sun protection, can effectively manage this photosensitive eruption.
Area of Science:
- Pediatric Dermatology
- Neonatal Immunology
- Maternal-Fetal Medicine
Background:
- Infantile periorbital eruptions can present as photosensitive facial dermatitis.
- These rashes may be associated with maternal autoimmune diseases, particularly Sjögren syndrome.
- Anti-SSA antibodies are frequently detected in affected infants and their mothers.
Observation:
- Three infant girls presented with similar annular, polycyclic, erythematous, scaly plaques on the face, predominantly periorbital.
- All infants had positive anti-SSA antibodies, with one also testing positive for anti-SSB antibodies.
- Maternal evaluations revealed positive anti-SSA antibodies in two mothers, with one having a history of Sjögren syndrome.
Findings:
- Infant facial eruptions showed a strong association with maternal anti-SSA antibody positivity.
- Skin lesions responded to topical treatments such as desonide cream or tacrolimus ointment, combined with sun protection.
- Cardiac evaluations and electrocardiograms in the infants were normal, ruling out significant congenital heart block.
Implications:
- Maternal anti-SSA antibody screening should be considered in infants with persistent photosensitive facial rashes.
- Prompt diagnosis and management, including photoprotection, can lead to favorable dermatological outcomes.
- This finding underscores the importance of understanding transplacental antibody transfer in neonatal skin conditions.
Abstract:
The authors report 3 infant girls with a similar periorbital eruption. A 5-month-old infant girl presented with a 3-month history of a photosensitive facial eruption that had failed to respond to topical 1% hydrocortisone cream. The otherwise healthy infant was the product of a term pregnancy. The 25-year-old mother was in good health except for occasional arthralgias. She had 2 other healthy children. Physical examination revealed a well-developed, well-nourished infant with annular, polycyclic, erythematous scaly plaques involving the cheeks and periorbital region (Figure 1). Potassium hydroxide scraping from the face was negative for dermatophyte and yeast. Laboratory studies revealed normal complete blood cell count, normal liver function tests, strongly positive anti-SSA antibody at 118 units (>80 units, strongly positive), and a negative anti-SSB antibody. Cardiac examination and electrocardiogram (ECG) were also normal. Laboratory evaluation of the mother revealed a positive anti-SSA at 158 units and mild anemia, but anti-SSB, anti-Smith, U1RNP, and anti-Scl-70 antibodies were all negative. Within 4 months the facial eruption cleared completely with the use of desonide cream 0.05% applied twice a day and sun protection. A 6-month-old girl was referred for dermatitis that began on the trunk and face at about 2 months of age. Although the truncal component resolved after 2 months, the facial dermatitis persisted. The infant was in good health and was the first-born child. The mother was known to have Sjögren syndrome. Physical examination revealed the characteristic erythematous, annular, scaling, polycyclic plaques along the forehead, periorbital cheeks, and eyelids (Figure 2). Laboratory evaluation of the infant revealed normal complete blood cell count, liver function tests, and chemistry profile. Anti-SSA antibody was positive at >6 units (reference, <1 unit) and anti-SSB antibody was positive at 2.84 units (reference, <1 unit). U1RNP antibody was negative. Cardiac examination and ECG were normal. The skin abnormalities cleared completely in 6 weeks with the topical application of tacrolimus 0.03% ointment and sun protection. A 5-month-old girl presented with a 2-month history of a persistent facial dermatitis. The infant was in good health and was the product of a healthy first pregnancy and delivery. The mother was in good health. Physical examination of the infant revealed erythematous, annular, polycyclic periorbital patches (Figure 3). Laboratory evaluation revealed positive SSA and SSB antibodies (units unavailable) and normal complete blood cell count, liver function tests, and chemistry profile. Cardiac examination and ECG were normal. The mother's laboratory results were also positive for anti-SSA and anti-SSB antibodies (units unavailable). The infant's facial eruption resolved without specific treatment.