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Annular Rash on a Newborn Keith A JFP_02.06_PhotoRnds.Final 1/23/06 1:31 PM Page 127 PHOTO ROUNDS Anne-Marie Warner, MD, Annular rash on a newborn Keith A. Frey, MD, and Suzanne Connolly, MD Departments of Family Medicine and Dermatology, Mayo Clinic Arizona, full-term, healthy female new- suggestive of left ventricular hypertro- Scottsdale born was delivered via cesarean phy. An echocardiogram was normal. A section because the labor did not Follow-up office visits for com- adequately progress. The mother, age 33 mon newborn feeding problems years and of Asian ancestry, had a signif- demonstrated consistent weight gain icant medical and obstetrical history: and normal vital signs, including chronic hepatitis B carrier without cir- heart rate and facial milia. However, rhosis, cutaneous lupus erythematosus by age 4 weeks an erythematous erup- (positive anti-Ro and anti-La antibodies), tion extending from the frontal scalp and a positive group B streptococcal and forehead to the cheek area had recto-vaginal culture at 35 weeks’ gesta- developed (FIGURES 1 AND 2). tion. The mother received 4 doses of intravenous ampicillin during labor. The infant’s initial hospital course ■ What is the differential was complicated by a transient and diagnosis? otherwise asymptomatic bradycardia. An electrocardiogram (ECG) confirmed a heart rate of 96 with normal interval ■ What tests should be done parameters, but there were changes to make the diagnosis? FIGURE 1 Rash on a newborn’s face ... FIGURE 2 ...and on the scalp FEATURE EDITOR Richard P. Usatine, MD University of Texas Health Sciences Center at San Antonio CORRESPONDENCE Keith A. Frey, MD, Department of Family Medicine, Mayo Clinic Arizona, 13737 North 92nd Discrete annular erythematous lesions with Similar lesions involving the light-exposed Street, Scottsdale, AZ 85260. secondary scaling are scattered over the face. areas of the scalp. E-mail: [email protected] www.jfponline.com VOL 55, NO 2 / FEBRUARY 2006 127 JFP_02.06_PhotoRnds.FinalREV 1/24/06 3:08 PM Page 128 PHOTO ROUNDS ■ Diagnosis: Neonatal lupus group of disorders that are characterized This infant has neonatal lupus erythemato- by chronicity without autoantibodies. sus, a rare syndrome in which maternal autoantibodies are passively transferred to the baby and cause cutaneous lesions or ■ Causes and pathophysiology isolated congenital heart block. The skin of neonatal lupus rash generally appears a few days to weeks Neonatal lupus is caused by the IgG after birth, typically after sun exposure, autoantibodies anti-Ro/SSA and anti- and shows well-demarcated erythematous, La/SSB, which pass from the mother to the scaling patches that are often annular and fetus via the placenta, although anti-U1- predominately on the scalp, neck, or face. RNP antibodies can sometimes cause the It is self-limited and generally resolves cutaneous manifestations of neonatal without scarring by 6 to 7 months of age. lupus.2 The mother may or may not demonstrate features of connective tissue disease at the time of birth. Lupus has a ■ Differential diagnosis higher incidence in Asian women than in The differential diagnosis of neonatal the overall US population. lupus syndrome includes annular urticaria, Possible symptoms in the neonate tinea corporis, seborrheic dermatitis, include a characteristic skin eruption or erythema annulare centrifugum, familial congenital heart block. Neonatal lupus annular erythema, erythema multiforme, accounts for 85% of cases of congenital systemic lupus erythematosus, pityro- complete heart block diagnosed in utero or sporum infection, and photosensitive gen- in the neonatal period.3 The incidence of odermatoses.1 These can be differentiated complete heart block in offspring of from neonatal lupus by several defining women with anti-Ro/SSA or anti-La/SSB characteristics: antibodies is about 2%. The incidence of • Annular urticaria is transient and pruritic complete heart block in infants with cuta- • In tinea corporis, a KOH prep reveals neous neonatal lupus is 15% to 30%. FAST TRACK numerous branching hyphae Incomplete heart block, which may All infants born • Seborrheic dermatitis is characterized by progress to complete heart block, may also greasy scales over red, inflamed skin and occur. A less common and self-limited to women with distributed in facial and body folds sinus bradycardia has also been described lupus should have • Erythema annulare centrifugum usually in fetuses.4 an ECG to detect affects the trunks and legs; the character- Other manifestations of neonatal istic rings enlarge daily lupus include thrombocytopenia, aplastic heart block; • Familial annular erythema is generally anemia, hepatobiliary dysfunction, and if abnormal, found on the back and shoulders and central nervous system vasculopathy. refer to a pediatric accompanied by a family history of cardiologist similar lesions in infancy • Erythema multiforme is more often on ■ Diagnosis and treatment extensor surfaces and is uncommon in Diagnosis is based on physical findings in infancy an infant aged <6 months and detection of • Systemic lupus erythematosus should be anti-Ro/SSA and anti-La/SSB antibodies in suspected if the infant has the clinical the child or mother. It should be suspected manifestations or positive serological in children with congenital heart block as it tests while autoantibodies are absent in is responsible for over 85% of cases. the mother All infants diagnosed with neonatal • Pityrosporum infection can be diagnosed lupus erythematosus or born to women by hyphae and spores (“spaghetti and with anti-Ro/SSA or anti-La/SSB anti- meatballs”) on KOH preparation bodies should have an ECG to detect • Photosensitive genodermatoses are a rare heart block. If this is abnormal, referral 128 VOL 55, NO 2 / FEBRUARY 2006 THE JOURNAL OF FAMILY PRACTICE JFP_02.06_PhotoRnds.FinalREV 1/25/06 2:52 PM Page 129 Annular rash on a newborn ▲ to a pediatric cardiologist is warranted. infant had anti-Ro, anti-La, and anti-RNP All pregnant women with anti-Ro/SSA levels of 128.3, 150.3, and 128, respective- or anti-La/SSB antibodies should under- ly. Normal range is 0 to 19 for each go regular fetal echocardiography to autoantibody. Her mother’s autoantibody detect heart block. The initiation of dex- levels were similarly abnormal at 138.1, amethasone or plasmapheresis as a pre- 158.8, and 169.1. ventative measure against congenital The patient was referred to a pediatric heart block in high-risk pregnant women cardiologist for follow-up; results of a sec- is currently under consideration but is ond echocardiogram and ECG were nor- not yet justified.3 mal. The infant was seen again at 7 weeks; Treatment includes photoprotection cutaneous lesions were present but some- (avoiding sunlight using clothing and other what improved, and she was otherwise protective measures) and time, as nearly all doing well. Given the high probability of cases resolve within 6 months without lupus occurring in future pregnancies, the scarring. Mild topical steroids may be mother received appropriate education. ■ helpful. It is important to note that chil- dren with neonatal lupus may be at higher REFERENCES risk of developing autoimmune disorders 1. Silverman ED. Neonatal lupus. In Cassidy JT, Pett 2 or rheumatic disease later in life. RE (eds): Textbook of Pediatric Rheumatology. Philadelphia: WB Saunders; 2001:456. 2. Habif TP. Clinical Dermatology. Edinburgh: Mosby; ■ Outcome 2004. 3. Buyon JP, Clancy RM. Neonatal lupus: Basic research For this patient, the skin lesions were and clinical perspective. Rheum Dis Clin North Am scraped with the side of a microscope slide 2005; 31:299–313. and KOH with DMSO was used to dis- 4. Askanase AD, Friedman DM, Copel J, et al. Spectrum and progression of conduction abnormalities in solve the epithelial cells. The preparation infants born to mothers with anti-SSA/Ro-SSB/La anti- was negative for fungal elements.This bodies. Lupus 2002; 11:145. Free 2.5 CME Credits Act Now! March 2006 Available through Supplement available at JFPonline.com This supplement to The Journal of Family Practice was supported Sponsored by the University of by a grant from Ethicon Endo-Surgery, Inc. It has been edited and Cincinnati College of Medicine peer reviewed by The Journal of Family Practice. .
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