Question:
A 2-week-old infant presents with a photosensitive annular erythematous rash on the face. ECG reveals complete heart block. Which is the most likely diagnosis?
A 2-week-old infant presents with a photosensitive annular erythematous rash on the face. ECG reveals complete heart block. Which is the most likely diagnosis?
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Remember: While cutaneous, hepatic, and hematologic manifestations of neonatal lupus are transient and resolve as maternal antibodies wane, congenital heart block is permanent and usually requires permanent pacemaker placement.
Updated On: Sep 3, 2026
- Congenital CMV infection
- Neonatal lupus
- Atopic dermatitis
- Erythema multiforme
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The Correct Option is B
Solution and Explanation
Concept:
Neonatal lupus erythematosus (NLE) is a passively acquired autoimmune syndrome occurring in infants born to mothers with autoantibodies against Ro/SSA, La/SSB, or rarely U1-RNP.
The hallmark clinical triad consists of cutaneous lesions, congenital conduction abnormalities (most characteristically third-degree AV block), and transient hepatobiliary or hematologic disturbances.
Explanation:
• Cutaneous manifestations typically appear in the first few weeks of life and classically present as annular, polycyclic, erythematous, scaly plaques predominantly located in periorbital (giving a "raccoon-eye" or "owl-eye" appearance) and facial regions following sun exposure.
• The cutaneous rash is transient and gradually resolves within 6 to 9 months as maternal IgG antibodies are cleared from the infant's circulation, leaving minimal or no scarring.
• Cardiac involvement, unlike cutaneous manifestations, is permanent and irreversible because antibody-mediated inflammation triggers fibrosis and calcification of the fetal conduction system.
• Congenital cytomegalovirus (CMV) infection presents with sensorineural hearing loss, periventricular calcifications, and microcephaly, but not heart block.
• Atopic dermatitis typically appears after 2--3 months of age, involves flexural or extensor surfaces, and is not associated with complete heart block.
Final Answer:
The constellation of a photosensitive annular facial rash combined with complete heart block in a neonate is diagnostic of neonatal lupus erythematosus.
Neonatal lupus erythematosus (NLE) is a passively acquired autoimmune syndrome occurring in infants born to mothers with autoantibodies against Ro/SSA, La/SSB, or rarely U1-RNP.
The hallmark clinical triad consists of cutaneous lesions, congenital conduction abnormalities (most characteristically third-degree AV block), and transient hepatobiliary or hematologic disturbances.
Explanation:
• Cutaneous manifestations typically appear in the first few weeks of life and classically present as annular, polycyclic, erythematous, scaly plaques predominantly located in periorbital (giving a "raccoon-eye" or "owl-eye" appearance) and facial regions following sun exposure.
• The cutaneous rash is transient and gradually resolves within 6 to 9 months as maternal IgG antibodies are cleared from the infant's circulation, leaving minimal or no scarring.
• Cardiac involvement, unlike cutaneous manifestations, is permanent and irreversible because antibody-mediated inflammation triggers fibrosis and calcification of the fetal conduction system.
• Congenital cytomegalovirus (CMV) infection presents with sensorineural hearing loss, periventricular calcifications, and microcephaly, but not heart block.
• Atopic dermatitis typically appears after 2--3 months of age, involves flexural or extensor surfaces, and is not associated with complete heart block.
Final Answer:
The constellation of a photosensitive annular facial rash combined with complete heart block in a neonate is diagnostic of neonatal lupus erythematosus.
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