Anne-Marie Warner, MD,

Keith A. Frey, MD, and

Suzanne Connolly, MDDepartments of FamilyMedicine and Dermatology,Mayo Clinic Arizona,Scottsdale

VOL 55, NO 2 / FEBRUARY 2006 127w w w. j f p o n l i n e . c o m

F E A T U R E E D I T O R

Richard P. Usatine, MDUniversity of Texas Health Sciences Center at San Antonio

C O R R E S P O N D E N C E

Keith A. Frey, MD,Department of FamilyMedicine, Mayo ClinicArizona, 13737 North 92ndStreet, Scottsdale, AZ 85260.E-mail: frey.keith@mayo.edu

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Afull-term, healthy female new-born was delivered via cesareansection because the labor did not

adequately progress. The mother, age 33years and of Asian ancestry, had a signif-icant medical and obstetrical history:chronic hepatitis B carrier without cir-rhosis, cutaneous lupus erythematosus(positive anti-Ro and anti-La antibodies),and a positive group B streptococcalrecto-vaginal culture at 35 weeks’ gesta-tion. The mother received 4 doses ofintravenous ampicillin during labor.

The infant’s initial hospital coursewas complicated by a transient and otherwise asymptomatic bradycardia. Anelectrocardiogram (ECG) confirmed aheart rate of 96 with normal intervalparameters, but there were changes

suggestive of left ventricular hypertro-phy. An echocardiogram was normal.

Follow-up office visits for com-mon newborn feeding problemsdemonstrated consistent weight gainand normal vital signs, includingheart rate and facial milia. However,by age 4 weeks an erythematous erup-tion extending from the frontal scalpand forehead to the cheek area haddeveloped (FIGURES 1 AND 2).

■ What is the differentialdiagnosis?

■ What tests should be doneto make the diagnosis?

Annular rash on a newborn

FIGURE 1 Rash on a newborn’s face ...

Discrete annular erythematous lesions with

secondary scaling are scattered over the face.

FIGURE 2 ...and on the scalp

Similar lesions involving the light-exposed

areas of the scalp.

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All infants born to women withlupus should havean ECG to detectheart block; if abnormal, refer to a pediatriccardiologist

128 VOL 55, NO 2 / FEBRUARY 2006 THE JOURNAL OF FAMILY PRACTICE

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FAST TRACK

■ Diagnosis: Neonatal lupusThis infant has neonatal lupus erythemato-sus, a rare syndrome in which maternalautoantibodies are passively transferred tothe baby and cause cutaneous lesions orisolated congenital heart block. The skinrash generally appears a few days to weeksafter birth, typically after sun exposure,and shows well-demarcated erythematous,scaling patches that are often annular andpredominately on the scalp, neck, or face.It is self-limited and generally resolveswithout scarring by 6 to 7 months of age.

■ Differential diagnosisThe differential diagnosis of neonatallupus syndrome includes annular urticaria,tinea corporis, seborrheic dermatitis, erythema annulare centrifugum, familialannular erythema, erythema multiforme,systemic lupus erythematosus, pityro-sporum infection, and photosensitive gen-odermatoses.1 These can be differentiatedfrom neonatal lupus by several definingcharacteristics:• Annular urticaria is transient and pruritic• In tinea corporis, a KOH prep reveals

numerous branching hyphae • Seborrheic dermatitis is characterized by

greasy scales over red, inflamed skin anddistributed in facial and body folds

• Erythema annulare centrifugum usuallyaffects the trunks and legs; the character-istic rings enlarge daily

• Familial annular erythema is generallyfound on the back and shoulders andaccompanied by a family history of similar lesions in infancy

• Erythema multiforme is more often onextensor surfaces and is uncommon ininfancy

• Systemic lupus erythematosus should besuspected if the infant has the clinicalmanifestations or positive serologicaltests while autoantibodies are absent inthe mother

• Pityrosporum infection can be diagnosedby hyphae and spores (“spaghetti andmeatballs”) on KOH preparation

• Photosensitive genodermatoses are a rare

group of disorders that are characterizedby chronicity without autoantibodies.

■ Causes and pathophysiologyof neonatal lupus

Neonatal lupus is caused by the IgGautoantibodies anti-Ro/SSA and anti-La/SSB, which pass from the mother to thefetus via the placenta, although anti-U1-RNP antibodies can sometimes cause thecutaneous manifestations of neonatallupus.2 The mother may or may notdemonstrate features of connective tissuedisease at the time of birth. Lupus has ahigher incidence in Asian women than inthe overall US population.

Possible symptoms in the neonateinclude a characteristic skin eruption orcongenital heart block. Neonatal lupusaccounts for 85% of cases of congenitalcomplete heart block diagnosed in utero orin the neonatal period.3 The incidence ofcomplete heart block in offspring ofwomen with anti-Ro/SSA or anti-La/SSBantibodies is about 2%. The incidence ofcomplete heart block in infants with cuta-neous neonatal lupus is 15% to 30%.Incomplete heart block, which mayprogress to complete heart block, may alsooccur. A less common and self-limitedsinus bradycardia has also been describedin fetuses.4

Other manifestations of neonatallupus include thrombocytopenia, aplasticanemia, hepatobiliary dysfunction, andcentral nervous system vasculopathy.

■ Diagnosis and treatmentDiagnosis is based on physical findings inan infant aged <6 months and detection ofanti-Ro/SSA and anti-La/SSB antibodies inthe child or mother. It should be suspectedin children with congenital heart block as itis responsible for over 85% of cases.

All infants diagnosed with neonatallupus erythematosus or born to womenwith anti-Ro/SSA or anti-La/SSB anti-bodies should have an ECG to detectheart block. If this is abnormal, referral

JFP_02.06_PhotoRnds.FinalREV 1/24/06 3:08 PM Page 128

to a pediatric cardiologist is warranted.All pregnant women with anti-Ro/SSAor anti-La/SSB antibodies should under-go regular fetal echocardiography todetect heart block. The initiation of dex-amethasone or plasmapheresis as a pre-ventative measure against congenitalheart block in high-risk pregnant womenis currently under consideration but isnot yet justified.3

Treatment includes photoprotection(avoiding sunlight using clothing and otherprotective measures) and time, as nearly allcases resolve within 6 months withoutscarring. Mild topical steroids may behelpful. It is important to note that chil-dren with neonatal lupus may be at higherrisk of developing autoimmune disordersor rheumatic disease later in life.2

■ OutcomeFor this patient, the skin lesions werescraped with the side of a microscope slideand KOH with DMSO was used to dis-solve the epithelial cells. The preparationwas negative for fungal elements.This

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infant had anti-Ro, anti-La, and anti-RNPlevels of 128.3, 150.3, and 128, respective-ly. Normal range is 0 to 19 for eachautoantibody. Her mother’s autoantibodylevels were similarly abnormal at 138.1,158.8, and 169.1.

The patient was referred to a pediatriccardiologist for follow-up; results of a sec-ond echocardiogram and ECG were nor-mal. The infant was seen again at 7 weeks;cutaneous lesions were present but some-what improved, and she was otherwisedoing well. Given the high probability oflupus occurring in future pregnancies, themother received appropriate education. ■

R E F E R E N C E S

1. Silverman ED. Neonatal lupus. In Cassidy JT, Pett RE (eds): Textbook of Pediatric Rheumatology.Philadelphia: WB Saunders; 2001:456.

2. Habif TP. Clinical Dermatology. Edinburgh: Mosby;2004.

3. Buyon JP, Clancy RM. Neonatal lupus: Basic researchand clinical perspective. Rheum Dis Clin North Am2005; 31:299–313.

4. Askanase AD, Friedman DM, Copel J, et al. Spectrumand progression of conduction abnormalities ininfants born to mothers with anti-SSA/Ro-SSB/La anti-bodies. Lupus 2002; 11:145.

Annular rash on a newborn ▲

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