eMedicine Specialties > Pediatrics: General Medicine > Rheumatology

Neonatal Lupus and Cutaneous Lupus Erythematosus in Children

Author: Jeffrey P Callen, MD, Professor of Medicine, Chief, Division of Dermatology, University of Louisville School of Medicine
Contributor Information and Disclosures

Updated: Aug 18, 2008

Introduction

Background

Lupus erythematosus (LE) is a rare condition in children. Most cases take the form of systemic lupus erythematosus (SLE). This article discusses cutaneous lesions of lupus in neonates and children, as well as the relationship of these lesions to systemic disease. Specifically, this article addresses (1) neonatal LE (NLE), which usually manifests as nonscarring, non–atrophy-producing lesions known as subacute cutaneous LE (SCLE), and (2) cutaneous lesions of lupus erythematosus in children and adolescents.

Pathophysiology

Neonatal lupus erythematosus is thought to be related to circulating maternal autoantibodies; however, most infants exposed to these antibodies in utero do not develop disease. In one study, serum immunoglobulin G (IgG) from the mother of a neonate with congenital heart block (CHB) inhibited L-type calcium channels in a rat heart model.1 Additionally, induction of apoptosis in cultured cardiocytes has been demonstrated to result in the expression of Ro/La antigens on the cell surface. However, the antibodies associated with heart block and with cutaneous disease are believed to be different, with the Ro (SS-A) against the 52/60-kd protein associated with heart block, and the La (SS-B) against the 50-kd protein associated with cutaneous disease.

However, only some neonates exposed to these antibodies develop complications; therefore, other factors must be involved. These may include genetic predisposition, viral infection, and other unknown factors. The risk of neonatal lupus erythematosus or CHB developing in a woman who tests positive for Ro/SS-A who has never had a child with neonatal lupus erythematosus or CHB is less than 1%, whereas the risk for a mother who has had an affected infant is roughly 25%.

Lupus erythematosus of childhood is related to genetic factors, but other environmental events must also be involved. Lupus erythematosus in childhood may affect the skin or may manifest as systemic lupus erythematosus and affect any organ system in the body, most commonly the kidneys, joints, and blood. Neonatal lupus erythematosus may affect the skin, heart, liver, blood-forming elements, CNS, or the spleen.

Frequency

United States

Neonatal lupus erythematosus occurs in 1 of every 20,000 live births. Lupus erythematosus of childhood occurs in 0.6 of every 100,000 children annually.

Mortality/Morbidity

Neonatal lupus erythematosus has significant associated morbidity and mortality when the heart is affected. In patients with cardiac involvement, neonatal lupus erythematosus may manifest as complete or incomplete CHB. Heart block is evident in utero or at birth. In children who are severely affected, a pacemaker is frequently needed because sudden death or heart failure may occur. However, many children with CHB may be relatively asymptomatic until adolescence, when they begin to exercise. At this time, they may develop syncope and require a pacemaker implantation.

Most patients with neonatal lupus erythematosus of the skin, liver, or blood have transient disease that spontaneously resolves within 4-6 months. Children rarely develop systemic lupus erythematosus later in life. Siblings of affected individuals also have a risk of developing systemic lupus erythematosus later in life.

Morbidity and mortality of systemic lupus erythematosus of childhood depend on the organ systems affected. If the kidneys are affected, renal failure may occur. Joint disease does not lead to deformity but may be debilitating. Disease of the skin may lead to scar formation; however, in isolation, it is associated with a good prognosis.

Race

No racial predilection has been observed. However, lupus erythematosus of childhood appears to be more common in African Americans, Latin Americans, and Asian children (3:1 ratio in all races compared with whites).

Sex

Neonatal lupus erythematosus of the heart affects girls more often than boys (female-to-male ratio of 2:1). Neonatal lupus erythematosus of the skin affects girls more often than boys (female-to-male ratio of 3:1). Prepubertal female-to-male ratios have been reported to be between 1:1 and 3:1, whereas the ratio in postpubertal children is between 8:1 and 10:1.

Age

Neonatal lupus erythematosus affects children aged 0-6 months, whereas lupus erythematosus of childhood affects prepubertal and postpubertal children.

Clinical

History

  • Neonatal lupus erythematosus
    • The mother usually discovers neonatal lupus erythematosus (NLE) that affects the skin shortly after birth. In some instances, the mother notes that the infant is sensitive to sunlight.
    • Neonatal lupus erythematosus that affects the heart is usually noted upon physical examination at birth but may be recognized with ultrasonography in utero.
    • Mothers may be asymptomatic or have symptoms of lupus erythematosus (LE) or Sjögren syndrome. When carefully questioned, they may report dry eyes, arthralgia, myalgia, or arthritis. A recent report linked the presence of hypothyroidism in mothers with Ro with an increased risk of CHB.
  • Lupus erythematosus of childhood
    • Photosensitivity, arthritis, arthralgia, and fever may be the presenting symptoms. The patient may also report having a rash.
    • Adults with discoid lupus erythematosus (DLE) lesions have a low risk of systemic disease; however, risk of systemic disease and progression to systemic involvement appears to be greater in children, with one report suggesting rates of 50%. Therefore, question children with discoid lupus erythematosus or subacute cutaneous lupus erythematosus (SCLE) lesions about symptoms of pleuritis, pericarditis, and neurologic or renal involvement.

Physical

  • Cutaneous findings in neonatal lupus erythematosus
    • Annular erythematous plaques with a slight scale characterize neonatal lupus erythematosus. Atrophic lesions may develop; however, over time, even these lesions leave little residual change. These lesions are usually not present at birth but may become evident shortly afterward, particularly in infants exposed to light therapy.
    • Telangiectasia is often prominent and is the sole cutaneous manifestation reported in some patients.
    • Dyspigmentation is frequent, but, with time, this change spontaneously resolves.
    • Cutaneous lesions usually resolve. They are present at birth or shortly after and rarely appear when the infant is older than 6 months. The head and neck are the most commonly affected areas, but lesions may also appear on the arms and trunk.
  • Cardiac disease of neonatal lupus erythematosus
    • Complete CHB is the usual finding, but incomplete heart block is possible. This finding may be noted as a bradycardia in utero or during physical examination at birth.
    • Heart failure is a well-recognized complication during the neonatal period. Recently, incomplete heart block, as well as prolongation of the QT interval, has been noted.
  • Other manifestations of neonatal lupus erythematosus
    • Hepatosplenomegaly is an occasional transient finding. Transaminase elevation is often present.
    • Pneumonitis may manifest as tachypnea or tachycardia.
    • Thrombocytopenia may manifest as petechiae.
  • Findings in childhood lupus erythematosus: Lupus erythematosus in children may manifest as a polyarthritis, discoid lupus erythematosus or subacute cutaneous lupus erythematosus lesions, malar rash of lupus erythematosus, or photosensitive dermatitis.
  • Drug-induced lupus erythematosus
    • Drug-induced lupus erythematosus may develop in children and adolescents.
    • Recently, multiple cases of a lupus erythematosus–like disease have been reported in patients who are taking minocycline for acne. These patients often demonstrate fever and polyarthralgia or arthritis.
    • Recently, incomplete heart block, as well as prolongation of the QT interval, has been noted.

Causes

  • Neonatal lupus erythematosus is related to the transplacental passage of autoantibodies.
  • The cause of lupus erythematosus in children and adolescents is unknown, but genetic predisposition is likely.
  • Drugs, in particular minocycline, may be responsible for drug-induced disease.

More on Neonatal Lupus and Cutaneous Lupus Erythematosus in Children

Overview: Neonatal Lupus and Cutaneous Lupus Erythematosus in Children
Differential Diagnoses & Workup: Neonatal Lupus and Cutaneous Lupus Erythematosus in Children
Treatment & Medication: Neonatal Lupus and Cutaneous Lupus Erythematosus in Children
Follow-up: Neonatal Lupus and Cutaneous Lupus Erythematosus in Children
Multimedia: Neonatal Lupus and Cutaneous Lupus Erythematosus in Children
References
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References

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Further Reading

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Keywords

neonatal lupus, cutaneous lupus erythematosus, lupus erythematosus of childhood, neonatal lupus erythematosus, LE, NLE, cutaneous lesions, SCLE, subacute cutaneous lupus erythematosus, discoid lupus erythematosus, DLE, congenital heart block, CHB, telangiectasia, dyspigmentation, atrophic lesions, syncope, heart failure, Sjögren syndrome, arthritis, arthralgia, hepatosplenomegaly, pneumonitis, drug-induced lupus erythematosus, prolonged QT interval

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Contributor Information and Disclosures

Author

Jeffrey P Callen, MD, Professor of Medicine, Chief, Division of Dermatology, University of Louisville School of Medicine
Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and American College of Rheumatology
Disclosure: Amgen Honoraria Consulting; Abbott Honoraria Consulting; Electrical Optical Sciences Honoraria Consulting; Centocor Honoraria Consulting

Medical Editor

Barry L Myones, MD, Associate Professor, Departments of Pediatrics and Immunology, Pediatric Rheumatology Section, Baylor College of Medicine; Director of Research, Pediatric Rheumatology Center, Texas Children's Hospital
Barry L Myones, MD is a member of the following medical societies: American Academy of Pediatrics, American Association of Immunologists, American College of Rheumatology, American Heart Association, American Society for Microbiology, Clinical Immunology Society, and Texas Medical Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Pfizer Inc Stock Investment from broker recommendation; Avanir Pharma Stock Investment from broker recommendation

Managing Editor

Thomas JA Lehman, MD, FAAP, FACR, Clinical Professor of Pediatrics, Department of Pediatrics, Division of Pediatric Rheumatology, Weill-Cornell University; Chief, Hospital for Special Surgery
Thomas JA Lehman, MD, FAAP, FACR is a member of the following medical societies: PM American Allergy Society
Disclosure: Nothing to disclose.

CME Editor

Daniel Rauch, MD, FAAP, Director, Pediatric Hospitalist Program, Associate Professor, Department of Pediatrics, New York University School of Medicine
Daniel Rauch, MD, FAAP is a member of the following medical societies: Ambulatory Pediatric Association, American Academy of Pediatrics, and Society of Hospital Medicine
Disclosure: Baxter Honoraria Consulting; Pfizer Honoraria Consulting

Chief Editor

Barry L Myones, MD, Associate Professor, Departments of Pediatrics and Immunology, Pediatric Rheumatology Section, Baylor College of Medicine; Director of Research, Pediatric Rheumatology Center, Texas Children's Hospital
Barry L Myones, MD is a member of the following medical societies: American Academy of Pediatrics, American Association of Immunologists, American College of Rheumatology, American Heart Association, American Society for Microbiology, Clinical Immunology Society, and Texas Medical Association
Disclosure: Nothing to disclose.

 
 
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