Dermatology Question of the Week: Pediatric Problems
A 2-week-old girl is brought to the clinic for the rash pictured below.
She was born at term to a healthy mother after an uncomplicated pregnancy. The infant is feeding well and has no signs of respiratory distress or cyanosis. Which of the following is the most appropriate screening test for complications of this condition?
A. High-resolution chest CT
B. Electrocardiogram
C. Skin biopsy with direct immunofluorescence
D. Serum porphyrin levels
E. Esophageal manometry
Rationale:
This question evaluates the ability to recognize neonatal lupus erythematosus (NLE) and appropriately screen for congenital heart block, its most serious complication. NLE results from transplacental passage of maternal anti-Ro (SSA) and/or anti-La (SSB) antibodies. The rash typically appears within the first few weeks of life, particularly on the face and scalp, and is often photosensitive. The skin findings resolve spontaneously by 6–9 months as maternal antibodies degrade.
Correct Answer: B. Electrocardiogram
Congenital heart block is the most serious complication of NLE, occurring in 15–30% of affected neonates, and may be asymptomatic at birth. Electrocardiogram is the preferred first-line screening test to detect prolonged PR intervals or evidence of second- or third-degree heart block.
Incorrect answer choices:
A. High-resolution chest CT
HRCT is used to evaluate interstitial lung disease, which is a concern in systemic lupus erythematosus (SLE) but not neonatal lupus. CHB is the primary concern in NLE, not lung pathology.
C. Skin biopsy with direct immunofluorescence
A biopsy might show interface dermatitis with epidermal atrophy, but it is not necessary for diagnosis. Diagnosis of NLE is primarily clinical with serologic support (anti-Ro/SSA, anti-La/SSB), and biopsy does not assess systemic complications.
D. Serum porphyrin levels
Serum porphyrin testing is used to diagnose porphyria cutanea tarda (PCT), which presents with photosensitivity and blistering, but not annular plaques or congenital heart block.
E. Esophageal manometry
Esophageal dysmotility screening is useful in systemic sclerosis, but not neonatal lupus, which does not cause significant gastrointestinal involvement.
Additional reading at Weinberg's Color Atlas of Dermatology Section 19: Cutaneous Manifestations of Systemic Disease
I am a general dermatologist practicing at an academic medical center in Danville, PA. I see both adult and pediatric patients and also perform various surgical procedures. I have a special interest in Hidradenitis Suppurativa (HS) and am the founder of our HS specialty clinic where we treat patients medically and surgically (deroofing, excision, staged CO2 excision with marsupialization, Nd:YAG). I am actively involved in resident education and am passionate about medical education. Additional interests include autoimmune bullous disorders, dermatomyositis, and procedural dermatology.
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