Neonatal Lupus Erythematosus

Neonatal lupus erythematosus (NLE) is a rare autoimmune syndrome that affects 2% to 4% of infants born of mothers who are anti-Ro (SSA), anti-La (SSB), and/or anti-RNP antibody positive.
Most mothers do not show any signs or symptoms of connective tissue disease at the time of birth of the affected infant; however, Sjögren syndrome, systemic lupus erythematosus, or undifferentiated autoimmune syndrome does ultimately develop in some of these women.

NLE is an autoimmune condition caused by the passage of maternal antibodies to the neonate in utero.

NLE has a varied clinical presentation that includes a characteristic skin rash with or without systemic abnormalities and congenital complete heart block.
Most commonly, NLE manifests as a skin rash on the face (especially on forehead and periorbital), scalp, and extensor extremities, although lesions can occur anywhere.
The spectrum of skin findings includes annular erythematous, flat or edematous lesions with or without scale, variably sized erythematous patches, periorbital erythema (“owl's sign” or “raccoon eyes”), scaly atrophic macules and patches, or telangiectasias (Figs. 2.14 and 2.15).
Lesions can be present at birth or be delayed up to 20 weeks after birth, and have a mean age of onset of 6 weeks.
Skin lesions can be precipitated or worsened by sun exposure.
Thrombocytopenia or, less often, other hematologic abnormalities may be present. Liver dysfunction (elevated transaminases, cholestasis, or hyperbilirubinemia) occurs in 10% to 25% of infants with NLE and is usually transient.
Complete congenital heart block, the most serious potential manifestation of NLE, occurs in 2% to 4% of patients and is usually apparent at birth. First- and second-degree heart blocks, cardiomyopathy, or myocarditis have also been reported in up to 10% to 20% of patients.
There are isolated reports of neurologic abnormalities in children with NLE.

NLE should be suspected in infants with the typical appearance and distribution of the skin lesions.
Positive anti-Ro, anti-La, or anti-U1RNP antibodies in the infant and mother will confirm the diagnosis.
Patients with positive antibodies require a thorough physical examination, complete blood count, liver function test, and a cardiac evaluation to determine the full extent of the syndrome.

Differential Diagnosis

Urticaria

Tinea Corporis

Infantile Seborrheic Dermatitis (see below)

Ill-defined eczematous pink plaques with greasy yellow scale on face and scalp.
Occasionally brown adherent scale on scalp.
Responds quickly to low-potency topical steroids and/or topical antifungals.

Management

Skin lesions usually self-resolve by 6 to 12 months of age, concurrent with the clearance of maternal antibodies, and heal without sequelae.
Occasionally residual atrophy and telangiectasias persist.
Hematologic and hepatic complications also typically resolve when antibodies are cleared.
Skin lesions are treated with meticulous photoprotection, emollients, and low- to mid-potency topical steroids (desonide 0.05% ointment or fluocinolone 0.025% ointment).
Systemic abnormalities may require oral or IV corticosteroids or intravenous immunoglobulin.
Cardiac complications require management by a pediatric cardiologist. Complete heart block is irreversible and often requires pacemaker insertion.
Mothers of infants with NLE should be counseled that the risk of NLE in a subsequent child increases to 20%; consequently, they should have appropriate prenatal monitoring in subsequent pregnancies.

Helpful Hints

Periorbital erythema in a neonate should always prompt consideration of NLE.
Studies show that infants with NLE have a similar risk as nonaffected siblings for the development of future autoimmune disease.

Points to Remember

NLE presents with a characteristic skin eruption on photo-exposed areas at birth or shortly thereafter.
Positive anti-Ro, anti-La, and/or anti-U1RNP antibodies in maternal and infant serum will confirm the diagnosis of NLE.