Round, confluent patches on infant's face - Clinical Advisor

Round, confluent patches on infant’s face

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  • June 2015 Dermatology Clinic

    June 2015 Dermatology Clinic

A 5-week-old male presents with a 1-week history of cutaneous lesions affecting his scalp, face, and trunk. He has erythematous, round, confluent macules and patches on his face, most prominently around the eyes. Erythematous annular plaques with fine scales are noted on his trunk. Although he is distressed during examination, he appears well and shows no signs of infection. His mother reports a normal pregnancy and uncomplicated vaginal delivery. She denies health problems, but complains of dry mouth.


This The Clinical Advisor CME activity consists of 3 articles. To obtain credit, read Small, erythematous papules on the face, scalp, and neck and Pink-to-red scaly raised lesions. Then take the post-test here.


This The Clinical Advisor CME activity consists of 3 articles. To obtain credit, read Small, erythematous papules on the face, scalp, and neck and Pink-to-red scaly raised lesions. Then take the post-test here.Neonatal lupus erythematosus (NLE) is a passively transferred...

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This The Clinical Advisor CME activity consists of 3 articles. To obtain credit, read Small, erythematous papules on the face, scalp, and neck and Pink-to-red scaly raised lesions. Then take the post-test here.


Neonatal lupus erythematosus (NLE) is a passively transferred autoimmune condition that affects a minority of infants born to mothers who are positive for autoantibodies against Ro/SSA, La/SSB, or less commonly, U1-ribonucleoprotein (RNP).1,2 Affected infants manifest symptoms of systemic lupus erythematosus (SLE) such as cutaneous lesions, cardiac disease, hepatobiliary disease, and hematologic cytopenias.1,2,3 In most cases, only one system is affected, but any combination of organ involvement may occur. 


NLE is a rare condition that is usually benign and self-limited but may be associated with serious sequelae. The incidence of NLE is not precisely known but has been estimated to be 1 in 12,500 to 20,000 live births.1,2 There is a predilection for females (2:1) in the cutaneous form.1

Skin findings are present in more than half of infants affected by NLE.2 The lesions may be present at birth but are most commonly observed a few weeks after birth.1,2,4 Cutaneous NLE is characterized by erythematous annular plaques with slight central atrophy. The lesions do not scar, although dyspigmentation may occur. Discoid lesions, scaly atrophic patches, and telangiectasia have also been described.4 The eruption typically occurs on the face and scalp but may affect any area of skin.1,3 In many cases, facial lesions localize around the eyes, such that the confluent periorbital erythema imparts an “owl eye” appearance.1,3,4

Sun exposure has been noted to exacerbate or trigger the onset of lesions. However, as skin lesions may be seen at birth and may appear in photoprotected areas, ultraviolet (UV) light exposure is not a requirement for the eruption.1,2 New lesions do not occur after three months of life.4

Cutaneous lesions are expected to resolve spontaneously by ages 6 to 8 months, accompanying the clearance of maternal autoantibodies from the infant’s serum.1,2 In rare cases, there may be residual hypopigmentation, epidermal atrophy, and persistent telangiectasia at the affected sites.1,2 For most children, there is no evidence later in life that the lesions ever existed.


Biopsy of the skin lesions demonstrates findings that are identical to those in subacute cutaneous lupus in adults. The major histologic features are keratinocyte damage, vacuolar degeneration at the dermal-epidermal junction, and a superficial mononuclear cell infiltrate.1,2 Direct immunofluorescence shows immune deposits around the basal keratinocytes and along the basement membrane.2

Most infants with cutaneous lupus do not have extracutaneous involvement. The frequency of systemic findings has not been established. The most severe manifestation of NLE is third-degree heart block, which may be detected in utero between 18 and 24 weeks of gestation as arrhythmia or bradycardia and, unlike the other symptoms of NLE, is irreversible.1,2,3 NLE accounts for 80% of all congenital heart block.2 Cardiac disease in neonatal lupus is associated with high morbidity, with most infants requiring placement of a permanent pacemaker, and a significant mortality rate of 20% to 30%.2

Some infants with NLE experience hepatobiliary disease that presents in the form of fulminant liver failure, hepatomegaly, cholestasis with conjugated hyperbilirubinemia, or elevated liver enzymes.1,2,3 The most commonly noted hematologic abnormality is thrombocytopenia, which is transient and usually not problematic. Unlike in adult lupus, lymphopenia is not seen in NLE.


Although the exact pathogenesis of NLE is unclear, there is evidence that it involves the transplacental passage of maternal immunoglobulin G autoantibodies (against Ro and/or La), leading to fetal tissue damage.3,4 It is unknown how specific tissues such as the skin and heart are injured. Nearly all infants with NLE are positive for these maternal autoantibodies, but only 1% to 2% of infants born to mothers with anti-Ro autoantibodies develop the symptoms of NLE.1 Since a minority of neonates exposed to these autoantibodies develop NLE, other factors must contribute to the disease process. As mentioned previously, the disappearance of maternal autoantibodies from circulation correlates with diminished disease activity, excluding cardiac disease.


The mother is often asymptomatic at the time of delivery; if she has been diagnosed with an autoimmune condition, she typically has a different pattern of organ involvement than her child.1 Some asymptomatic mothers eventually develop signs and symptoms of autoimmune disease, primarily SLE or Sjögren’s syndrome. Detection of maternal Ro/La autoantibodies during pregnancy warrants close monitoring of the fetus via serial echocardiography to detect heart block during the second trimester.2

The main differential diagnoses of isolated polycyclic skin lesions in a neonate may be distinguished from NLE by several characteristics: urticaria is transient and pruritic, erythema annulare centrifugum mainly localizes to the lower extremities, tinea corporis has a positive potassium hydroxide preparation, and seborrheic dermatitis causes greasy scaling on the scalp with lesions affecting skin folds. Conditions such as congenital rubella, congenital syphilis, Bloom syndrome, and Rothmund-Thomson syndrome should also be considered.


The diagnosis of NLE is made by clinical examination and by confirmation of the presence of maternal anti-Ro or anti-La autoantibodies in the infant’s serum.1,4 Although skin biopsy may provide additional diagnostic evidence, it is rarely necessary.


The long-term prognosis of infants with cutaneous NLE is excellent, as the dermatologic and non-cardiac abnormalities resolve spontaneously when maternal autoantibodies are cleared from the infant’s circulation.1,3 However, infants who recover from NLE are at greater risk of developing autoimmunity and collagen vascular diseases later in life.1,2

Since the cutaneous lesions are asymptomatic and will resolve without long-lasting sequelae, the management of cutaneous NLE consists of observation and education on avoiding sunlight. Low-potency topical steroids may help to decrease the erythema.1 Residual telangiectasia may be treated by a vascular laser if there are cosmetic concerns. Due to the adverse effects of steroid therapy, systemic glucocorticoids should be reserved only for severe forms of NLE that affect multiple organs.


For the patient in our case, an electrocardiogram was obtained and found to be within normal limits. Routine laboratory tests on the patient was also within normal limits. After four months of observation and without intervention, the infant’s lesions disappeared. A rheumatologist evaluated the baby’s mother and determined a diagnosis of Sjögren’s syndrome. 


Julie Nguyen, BS, is a medical student and Maura Holcomb, MD, is a dermatology resident at Baylor College of Medicine in Houston.



This The Clinical Advisor CME activity consists of 3 articles. To obtain credit, read Small, erythematous papules on the face, scalp, and neck and Pink-to-red scaly raised lesions. Then take the post-test here.


References


  1. Lee LA. Cutaneous lupus in infancy and childhood. Lupus. 2010;19(9):1112-1117.

  2. Boh EE. Neonatal lupus erythematosus. Clin Dermatol. 2004;22(2):125-128.

  3. Wisuthsarewong W, Soongswang J, Chantorn R. Neonatal lupus 
erythematosus: Clinical character, investigation, and outcome. 
Pediatr Dermatol. 2011;28(2):115-121.

  4. Moretti D, Cimaz R, Vannucci G, Marino A, De Martino M, Greco A. Cutaneous neonatal lupus: A case report and review of the literature. 
Int J Dermatol. 2014;53(12):1508-1512.


This The Clinical Advisor CME activity consists of 3 articles. To obtain credit, read Small, erythematous papules on the face, scalp, and neck and Pink-to-red scaly raised lesions. Then take the post-test here.


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