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Neonatal and Pediatric Lupus Erythematosus Treatment & Management

  • Author: Alisa N Femia, MD; Chief Editor: Lawrence K Jung, MD  more...
 
Updated: Jun 08, 2016
 

Approach Considerations

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

This child was one of a pair of fraternal twins. H 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.

Treatment should be supportive and depends on the specific manifestations present.

Treatment of cutaneous NLE is not required as lesions resolve without scarring. However, in cases in which therapy is desired, treatment includes mild topical corticosteroids, 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. Systemic corticosteroids may be used to treat or prevent cardiac NLE, but they are generally not recommended for established third-degree heart block as this condition is typically irreversible. While intravenous immunoglobulin (IVIG) has not been helpful in preventing congenital heart block, IVIG combined with corticosteroids administered prenatally to mothers may be helpful in preventing fetal cardiomyopathy or endocardial fibroelastosis.[31, 32] In severe cases, NLE 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 (LE). Consultations with specialists in dermatology, cardiology, rheumatology, nephrology, neurology, hepatology, immunology, and hematology may also be indicated.

Management of cutaneous and systemic LE in children is similar to management in adults and depends on specific disease manifestations. Therapy is directed towards any internal organ involvement and the prevention of dyspigmentation, disfigurement, and scarring as a result of cutaneous disease.

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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).[33] Mothers of such neonates, particularly neonates with congenital heart block, have at least a 2- to 3-fold increased risk of subsequent affected neonates. An estimated 15-25% of subsequent pregnancies are affected.[33] Therefore, carefully monitor subsequent pregnancies with serial ultrasonography and echocardiography, particularly at 18-24 weeks gestation.

Fluorinated systemic steroids may help prevent NLE. Intravenous immunoglobulin (IVIG) merits evaluation as a potential prophylactic approach in mothers who have previously had an affected child.[34] While neither of 2 recent studies demonstrated benefit in outcome IVIG,[35, 36] a recent study suggests that prenatal administration of IVIG along with corticosteroids may decrease the risk for fetal cardiomyopathy or endocardial fibroelastosis.[32]

The use of hydroxychloroquine for anti-Ro/SSA–positive mothers with systemic lupus erythematosus (SLE) has been associated with a lower rate of NLE during pregnancy.[37] In addition, aggregate multinational data have revealed that maternal use of hydroxychloroquine reduces the risk of NLE in subsequent pregnancies for mothers who are anti-Ro/SSA positive and have previously given birth to an infant with cardiac NLE, regardless of maternal health status.[38]

Consult an obstetrician with experience with high-risk pregnancies, and consider administration of prednisolone or fluorinated steroids during subsequent pregnancies to prevent NLE. In at least one instance, congenital heart block was prevented with the use of systemic corticosteroids beginning at 10 weeks’ gestation, azathioprine shortly thereafter, and plasmapheresis beginning at 18 weeks’ gestation.[39]

Children with NLE need continued follow-up, especially before adolescence and in cases in which the mother herself has an autoimmune disease.[40] Although the child may not be at increased risk of developing SLE, the development of some form of autoimmune disease in early childhood may be of concern.

Patients with NLE and 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 warranted.

Patients with cutaneous NLE do not require monitoring after lesions resolve. Although children with cutaneous disease may be more prone to develop lupus erythematosus (LE) later in life, this reflects genetic predisposition, rather than the occurrence of NLE earlier in life. Nonaffected siblings are also at risk for the development of SLE.

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Contributor Information and Disclosures
Author

Alisa N Femia, MD Assistant Professor, Ronald O Perelman Department of Dermatology, New York University Medical Center

Alisa N Femia, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, Society for Investigative Dermatology, Medical Dermatology Society, Rheumatologic Dermatology Society

Disclosure: Nothing to disclose.

Coauthor(s)

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, American College of Rheumatology

Disclosure: Received income in an amount equal to or greater than $250 from: XOMA; Biogen/IDEC; Novartis; Janssen Biotech, Abbvie, CSL pharma<br/>Received honoraria from UpToDate for author/editor; Received honoraria from JAMA Dermatology for associate editor and intermittent author; Received royalty from Elsevier for book author/editor; Received dividends from trust accounts, but I do not control these accounts, and have directed our managers to divest pharmaceutical stocks as is fiscally prudent from Stock holdings in various trust accounts include some pharmaceutical companies and device makers for i inherited these trust accounts; for: Celgene; Pfizer; 3M; Johnson and Johnson; Merck; Abbott Laboratories; AbbVie; Procter and Gamble; Amgen.

Ruth Ann Vleugels, MD, MPH Assistant Professor of Dermatology, Harvard Medical School; Associate Physician, Department of Dermatology, Brigham and Women's Hospital; Associate Physician, Department of Immunology and Allergy, Children's Hospital Boston

Ruth Ann Vleugels, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Rheumatology, American Medical Association, Society for Investigative Dermatology, Medical Dermatology Society, Dermatology Foundation

Disclosure: Nothing to disclose.

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, New York Academy of Sciences

Disclosure: Nothing to disclose.

Acknowledgements

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.

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.

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

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

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.

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.

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.

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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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