The patient is a 3-month-old, full-term male who presents with a rash on his head. The rash started one day prior to presentation on his forehead and spread to the rest of his head. Today, it developed a central clearing with surrounding redness. He has a history of sensitive skin since birth with patches of eczema and cradle cap. He treats these with Aquaphor and Honest Co. Cream; he has never been prescribed topical steroids for his rashes. Denies fever, cough, rhinorrhea, congestion, decreased appetite, diarrhea, and decreased urination. He had an uncomplicated birth history.
Skin: Large, serpiginous rash on the left forehead and scalp with central clearing and peripheral erythema as well as areas of erythematous plaques. He has some erythema of the left medial epicanthus. He also has a large erythematous patch at the base of his skull. The remainder of his skin is clear.
The image is of the cutaneous manifestation of neonatal lupus erythematosus. Neonatal lupus erythematosus is an autoimmune disease caused by transplacental passage of maternal autoantibodies to Sjögren’s syndrome A or B autoantigens (SS-A/SS-B). It can present with reversible changes including cutaneous lesions (most common, in up to 40% of patients), hepatobiliary disease, and cytopenias, which resolve once maternal autoantibodies have been cleared.
All infants that present with concern for neonatal lupus erythematosus should have screening labs performed to evaluate for hematologic, cardiac, and hepatobiliary involvement including a CBC with differential, liver enzymes, and antibody testing. In addition, an EKG is essential given that neonates can present with irreversible total atrioventricular heart block, which can present in utero or after birth.
The rash typically presents in the first few weeks of life but can present as late as 2-3 months of life (usually within 1-2 days of first sun exposure). Eighty percent of cases are not clear at birth and present in the first month of life. The rash appears as a coalescing rash with raised margins, with annular and discoid erythema involving the head in 95% of cases. It is often misdiagnosed as skin infections or eczema if the mom is asymptomatic. Fifty percent resolve by four months of life and 100% by one year.
Any neonate with a slow fetal heart rate or the postnatal diagnosis of atrioventricular heart block warrants immediate maternal testing for these autoantibodies. Most cardiac changes from neonatal lupus are diagnosed before 26 weeks gestation, with <20% later in pregnancy and 2% detected postnatally.