Neonatal and Pediatric Lupus Erythematosus Treatment & Management

  • Author: Jeffrey P Callen, MD; Chief Editor: Lawrence K Jung, MD   more...
 
Updated: Jul 14, 2011
 

Approach Considerations

Neonatal lupus erythematosus (NLE) that affects the skin, blood, spleen, or liver is usually self-limited and resolves without intervention within 2-6 months (see the image below).

This child was one of a pair of fraternal twins. HThis child was one of a pair of fraternal twins. Her sibling was not affected, although the mother and both infants had similar autoantibodies in their circulations. Eventually, the lesions seen here resolved and healed without sequelae.

Treatment of neonatal lupus erythematosus skin lesions includes mild steroids, antimalarial agents, and, possibly, laser treatment for residual telangiectasia. Photoprotection such as sunscreen and protective clothing is highly desirable, because solar exposure may precipitate skin lesions.

The type of treatment and the long-term prognosis for neonates with cardiac rhythm and conduction disturbances depends on the presence of underlying congenital heart abnormalities. In severe cases, neonatal lupus erythematosus that affects the heart may result in cardiac failure and death. A pacemaker is often necessary.

Consider transfer to a tertiary care center for all children and neonates with lupus erythematosus. Consultations with specialists in dermatology, cardiology, rheumatology, nephrology, neurology, immunology, and hematology may also be indicated.

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Prevention and Long-Term Monitoring

Observe mothers with positive autoantibodies and/or mothers who give birth to a child with neonatal lupus erythematosus (NLE).[17] Mothers of such neonates, particularly neonates with congenital heart block, have a 2-fold to 3-fold increased risk of subsequent affected neonates. An estimated 25% of subsequent pregnancies are affected.[17] Therefore, carefully monitor subsequent pregnancies with serial ultrasonography and echocardiography, particularly at 18-24 weeks’ gestation. Intravenous immunoglobulin merits evaluation as a potential prophylactic approach in mothers who have previously had an affected child.[18]

Neither of 2 recent studies demonstrated benefit in outcome from IVIG.[19, 20] However, the use of hydroxychloroquine for patients with SLE has been associated with a lower rate of NLE during pregnancy.[21]

Consult an obstetrician with experience with high-risk pregnancies, and consider administration of prednisolone or fluorinated steroids during subsequent pregnancies to prevent neonatal lupus erythematosus. In at least one instance, congenital heart block was prevented with the use of corticosteroids beginning at 10 weeks’ gestation, azathioprine shortly thereafter, and plasmapheresis beginning at 18 weeks’ gestation.[22]

Children with neonatal lupus erythematosus (NLE) need continued follow-up, especially before adolescence and if the mother herself has an autoimmune disease.[23] Although the child may not be at increased risk of developing systemic lupus erythematosus (SLE), the development of some form of autoimmune disease in early childhood may be of concern.

Patients with neonatal lupus erythematosus with cardiac involvement require regular monitoring to assess cardiac function and the need for a pacemaker. Thus, serial echocardiography to monitor for a prolonged PR interval may be a good idea.

Patients with cutaneous neonatal lupus erythematosus do not require monitoring after lesions resolve. However, strategies aimed at preventing disease before irrevocable scarring ensues are a high priority. Although children with cutaneous disease may be more prone to develop lupus erythematosus later in life, this reflects their genetic predisposition, not that they had neonatal lupus erythematosus. Their nonaffected siblings are also at risk for development of SLE.

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Contributor Information and Disclosures
Author

Jeffrey P Callen, MD  Professor of Medicine (Dermatology), 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 Consulting fee Consulting; Celgene Honoraria Safety Monitoring Committee; GSK - Glaxo Smith Kline Consulting fee Consulting; TenXBioPharma Consulting fee Safety Monitoring Committee

Coauthor(s)

Jack Grzybowski, MD  Staff Physician, Department of Pediatrics, University of Medicine and Dentistry of New Jersey-New Jersey Medical School

Jack Grzybowski, MD is a member of the following medical societies: Sigma Xi

Disclosure: Nothing to disclose.

Robert A Schwartz, MD, MPH  Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, University of Medicine and Dentistry of New Jersey-New Jersey Medical School

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi

Disclosure: Nothing to disclose.

Michael J Wells, MD  Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

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.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Janet Fairley, MD  Professor and Head, Department of Dermatology, University of Iowa, Roy J and Lucille A Carver College of Medicine

Janet Fairley, MD is a member of the following medical societies: American Academy of Dermatology, American Dermatological Association, American Federation for Medical Research, and Society for Investigative Dermatology

Disclosure: Nothing to disclose.

William D James, MD  Paul R Gross Professor of Dermatology, University of Pennsylvania School of Medicine; Vice-Chair, Program Director, Department of Dermatology, University of Pennsylvania Health System

William D James, MD is a member of the following medical societies: American Academy of Dermatology and Society for Investigative Dermatology

Disclosure: Elsevier Royalty Other

Chief Editor

Lawrence K Jung, MD  Chief, Division of Pediatric Rheumatology, Children's National Medical Center

Lawrence K Jung, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Rheumatology, Clinical Immunology Society, and New York Academy of Sciences

Disclosure: Nothing to disclose.

References

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Neonatal lupus erythematosus.
This child presented with petechial lesions, hepatosplenomegaly, and thrombocytopenia. Initially, he was thought to have histiocytosis (Letterer-Siwe disease); however, a skin biopsy revealed an interface dermatitis, and his mother had circulating autoantibodies.
This child was one of a pair of fraternal twins. Her sibling was not affected, although the mother and both infants had similar autoantibodies in their circulations. Eventually, the lesions seen here resolved and healed without sequelae.
 
 
 
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