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Pediatrics: Rheumatology > Rheumatology
Neonatal Lupus and Cutaneous Lupus Erythematosus in Children
Article Last Updated: Apr 4, 2007
AUTHOR AND EDITOR INFORMATION
Section 1 of 11  
Author: Jeffrey P Callen, MD, Chief, Division of Dermatology, Professor of Medicine (Dermatology), Department of Internal Medicine, University of Louisville School of Medicine
Jeffrey P Callen is a member of the following medical societies: American Academy of Dermatology, American College of Physicians, American College of Rheumatology, American Medical Association, Kentucky Medical Association, Noah Worcester Dermatological Society, Pacific Dermatologic Association, Society for Investigative Dermatology, Southeastern Dermatological Association, and Southern Medical Association
Editors: Barry L Myones, MD, Director of Research, Pediatric Rheumatology Center, Departments of Pediatrics & Immunology, Pediatric Rheumatology Section, Texas Children's Hospital at Houston; Associate Professor, Baylor College of Medicine; Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine.com, Inc; Thomas JA Lehman, MD, Clinical Professor of Pediatrics, Department of Pediatrics, Division of Pediatric Rheumatology, Weill-Cornell University; Chief, Hospital for Special Surgery; Daniel Rauch, MD, FAAP, Director, Pediatric Hospitalist Program, Associate Professor, Department of Pediatrics, New York University School of Medicine; Barry L Myones, MD, Director of Research, Pediatric Rheumatology Center, Departments of Pediatrics & Immunology, Pediatric Rheumatology Section, Texas Children's Hospital at Houston; Associate Professor, Baylor College of Medicine
Author and Editor Disclosure
Synonyms and related keywords:
neonatal lupus and cutaneous lupus erythematosus in children, 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
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 LE in children and adolescents.
Pathophysiology
NLE is thought to be related to circulating maternal autoantibodies; however, most infants exposed to these antibodies in utero do not develop disease. In recent studies, 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. 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 NLE or CHB developing in a woman who tests positive for Ro/SS-A who has never had a child with NLE or CHB is less than 1%, whereas the risk for a mother who has had an affected infant is roughly 25%. LE of childhood is related to genetic factors, but other environmental events must also be involved. LE in childhood may affect the skin or may manifest as SLE and affect any organ system in the body, most commonly the kidneys, joints, and blood. NLE may affect the skin, heart, liver, blood-forming elements, CNS, or the spleen.
Frequency
United States
NLE occurs in 1:20,000 live births. LE of childhood occurs in 0.6 of every 100,000 children annually.
Mortality/Morbidity
- NLE has significant associated morbidity and mortality when the heart is affected. In patients with cardiac involvement, NLE 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 NLE of the skin, liver, or blood have transient disease that spontaneously resolves within 4-6 months. Children rarely develop SLE later in life. Siblings of affected individuals also have a risk of developing SLE later in life.
- Morbidity and mortality of SLE 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, but, in isolation, it is associated with a good prognosis.
Race
- No racial predilection has been observed.
- LE of childhood appears to be more common in African Americans, Latin Americans, and Asian children (3:1 ratio of all races compared with whites).
Sex
- NLE of the heart affects girls more often than boys (female-to-male ratio of 2:1).
- NLE 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
- NLE affects children aged 0-6 months.
- LE of childhood affects prepubertal and postpubertal children.
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.
- NLE 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 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 LE (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 DLE or SCLE lesions about symptoms of pleuritis, pericarditis, and neurologic or renal involvement.
Physical
- Cutaneous findings in NLE
- Annular erythematous plaques with a slight scale characterize NLE. Atrophic lesions may develop, but, with 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 NLE
- 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 NLE
- 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 LE: LE in children may manifest as a polyarthritis, DLE or SCLE lesions, malar rash of LE, or photosensitive dermatitis.
- Drug-induced LE
- Drug-induced LE may develop in children and adolescents.
- Recently, multiple cases of an LE-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
- NLE is related to the transplacental passage of autoantibodies.
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- The cause of LE in children and adolescents is unknown, but genetic predisposition is likely.
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- Drugs, in particular minocycline, may be responsible for drug-induced disease.
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Atrioventricular Block, Second Degree
Atrioventricular Block, Third Degree, Congenital
Autoimmune Chronic Active Hepatitis
Juvenile Rheumatoid Arthritis
Mixed Connective Tissue Disease
Polyarteritis Nodosa
Sarcoidosis
Sjogren Syndrome
Systemic Lupus Erythematosus
Other Problems to be Considered
Juvenile dermatomyositis
Juvenile sarcoidosis
Drug eruptions
Viral or bacterial infections
Malignancy
Toxic exposures
Primary immunodeficiencies
Lab Studies
- Neonatal lupus erythematosus (NLE) is related to the anti-Ro (SS-A) antibody in more than 90% of patients. Occasionally, patients only have anti-La (SS-B) or anti-U1RNP antibodies.
- Test patients with NLE for cytopenia (CBC count) and liver enzyme elevations.
- Children in whom SLE is suspected should undergo a serologic evaluation including antinuclear antibody (ANA), anti-dsDNA, anti-Sm, anti-RNP, anti-Ro (SS-A), and anti-La (SS-B), as well as measurement of complement levels. Also test for other organ involvement, including a CBC count and tests of renal function.
Other Tests
- Electrocardiography demonstrates degree of heart block in a patient in whom NLE is suspected.
Procedures
- Skin biopsy is useful in patients with either NLE or cutaneous lesions of LE during childhood.
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Histologic Findings
Skin biopsy findings reveal interface dermatitis. Epidermal atrophy may be found. Inflammatory infiltrate may be so intense that bulla formation may develop histologically.
Medical Care
- Neonatal lupus erythematosus (NLE) that affects the skin, blood, spleen, or liver is usually self-limited and resolves without intervention within 2-6 months.
- In severe cases, NLE that affects the heart may result in cardiac failure and death. A pacemaker is often necessary.
- LE in children and adolescents requires therapy directed at the affected organ system.
- Treat skin conditions with sunscreen, topical corticosteroids, and antimalarial agents.
- Treat joint disease with nonsteroidal anti-inflammatory agents, physical therapy, antimalarial agents, and, occasionally, low-dose corticosteroids.
- Renal, CNS, or pulmonary disease usually requires moderate- or high-dose corticosteroids (1-2 mg/kg/d of prednisone or equivalent) with or without the addition of an immunosuppressive agent, such as azathioprine, methotrexate, mycophenolate mofetil, or cyclophosphamide.
Surgical Care
- No specific surgical care is indicated for patients with LE.
- Pacemaker placement may be needed in patients with NLE that affects the heart.
Consultations
- Dermatologist
- Rheumatologist
- Nephrologist
- Neurologist
- Immunologist
- Hematologist
Diet
- No specific diet is recommended.
Activity
- Limit activity only if the disease is active and base restrictions on the patient's abilities.
- Patients with skin disease should restrict their sun exposure.
Neonatal lupus erythematosus (NLE) does not require specific therapy. Some research suggests that infants from subsequent pregnancies are less likely to be affected by cardiac NLE if the mother is treated with prednisolone, dexamethasone, or betamethasone. Treatment for SLE in children and adolescents is based on the organ system involved. Treat skin disease with sunscreens, sun-protective measures, and topical corticosteroids. In patients with resistant symptoms, consider antimalarial drugs, such as hydroxychloroquine or chloroquine. In patients with renal or CNS involvement, consider high doses of prednisone with or without an immunosuppressive/cytotoxic agent. This treatment may result in control of the disease or even remission. Treat arthritis with physical therapy and oral nonsteroidal anti-inflammatory drugs (NSAIDs). Sunscreens are also useful in the treatment of cutaneous LE in children and adults. Sunscreens do not block all wavelengths of light; therefore, consider the preparation and its characteristics, rather than suggesting that any sunscreen is effective. In adult studies, the wavelengths of light responsible for induction of cutaneous LE are within the UV-B and UV-A range. Unfortunately, many sunscreens are labeled for UV-B protection only with the sun-protection factor (SPF). Patients and parents should be advised to apply sunscreen well before exposure and to use a sunscreen with a high SPF that provides a broad-spectrum (UV-A) coverage and is water-resistant. Reapplication after several hours is necessary. Encourage behavior modification of UV avoidance.
Drug Category: Topical corticosteroids
These agents are used to control cutaneous lesions. Select a specific agent based on treatment site and type of lesion. Facial skin is more prone to atrophy than skin of the scalp or hands; therefore, use a weaker agent on the face. Thick lesions may require more potent agents. In addition, treat hair-bearing areas with a lotion, gel, or foam instead of cream or ointment.
| Drug Name | Hydrocortisone (Dermacort, Westcort, CortaGel) |
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| Description | Lower potency topical steroids (0.5%, 1%, 2.5%) useful on face and intertriginous areas. Has mineralocorticoid and glucocorticoid effects that result in anti-inflammatory activity. |
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| Adult Dose | Apply tid initially; reduce as lesions remit |
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| Pediatric Dose | Administer as in adults |
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| Contraindications | Documented hypersensitivity; viral, fungal, and bacterial skin infections |
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| Interactions | None reported |
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| Pregnancy | C - Safety for use during pregnancy has not been established.
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| Precautions | Prolonged use, application over large surface areas, application of potent steroids, and occlusive dressings may increase systemic absorption of corticosteroids and may cause Cushing syndrome, reversible HPA axis suppression, hyperglycemia, and glycosuria |
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| Drug Name | Triamcinolone acetate (Aristocort, Kenalog) |
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| Description | Decreases inflammation by suppressing migration of PMNs and reversing capillary permeability. Moderate-potency topical steroid available in both ointment (0.1%) and cream (0.1%, 0.5%). |
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| Adult Dose | Apply tid initially; reduce as lesions remit |
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| Pediatric Dose | Administer as in adults |
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| Contraindications | Documented hypersensitivity; fungal, viral, and bacterial skin infections |
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| Interactions | None reported |
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| Pregnancy | C - Safety for use during pregnancy has not been established.
|
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| Precautions | Prolonged use may cause skin atrophy or steroid acne (less of a concern in prepubertal individuals); avoid use on face and intertriginous areas; limit use to 3 wk; systemic absorption of topical corticosteroids may cause Cushing syndrome, reversible HPA axis suppression, hyperglycemia, and glycosuria |
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| Drug Name | Clobetasol propionate (Temovate) |
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| Description | Superpotent topical steroid. Decreases inflammation by suppressing migration of PMNs and reversing capillary permeability. |
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| Adult Dose | Apply tid initially; reduce as lesions remit |
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| Pediatric Dose | Children: Not established
Adolescents: Administer as in adults |
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| Contraindications | Documented hypersensitivity; viral, fungal, and bacterial skin infections |
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| Interactions | None reported |
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| Pregnancy | C - Safety for use during pregnancy has not been established.
|
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| Precautions | Prolonged use may cause skin atrophy or steroid acne; avoid use on face and intertriginous areas; limit use to 3 wk and to older children and adults; systemic absorption of topical corticosteroids may cause Cushing syndrome, reversible HPA axis suppression, hyperglycemia, and glycosuria |
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| Drug Name | Betamethasone dipropionate (Alphatrex, Diprolene, Maxivate) |
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| Description | Superpotent topical steroid. Decreases inflammation by suppressing migration of PMNs and reversing capillary permeability. |
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| Adult Dose | Apply tid initially; reduce as lesions remit |
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| Pediatric Dose | Children: Not established
Adolescents: Administer as in adults |
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| Contraindications | Documented hypersensitivity; viral, fungal, and bacterial skin infections |
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| Interactions | None reported |
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| Pregnancy | C - Safety for use during pregnancy has not been established.
|
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| Precautions | Prolonged use may cause skin atrophy or steroid acne; avoid use on face and intertriginous areas; limit use to 3 wk and to older children and adults; systemic absorption of topical corticosteroids may cause Cushing syndrome, reversible HPA axis suppression, hyperglycemia, and glycosuria |
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Drug Category: Immunosuppressive agents
Patients with immune dysregulation and autoimmunity often benefit from immunosuppression. These drugs are useful in patients with skin disease that is unresponsive to topical agents and in patients with arthritis that does not respond to NSAIDs. These drugs are not needed in NLE but may be used in children with skin or joint disease of SLE.
| Drug Name | Hydroxychloroquine (Plaquenil) |
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| Description | Inhibits chemotaxis of eosinophils and locomotion of neutrophils and impairs complement-dependent antigen-antibody reactions.
Hydroxychloroquine sulfate 200 mg is equivalent to 155 mg hydroxychloroquine base and 250 mg chloroquine phosphate. |
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| Adult Dose | 200-400 mg (as sulfate salt)/d (3-7 mg/kg/d) PO |
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| Pediatric Dose | 3-5 mg base/kg/d PO; not to exceed 7 mg (as sulfate salt)/kg/d |
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| Contraindications | Documented hypersensitivity; G-6-PD deficiency; porphyria; psoriasis; retinal and visual field changes attributable to 4-aminoquinolones |
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| Interactions | Few reports; chloroquine may potentiate possible ocular toxicity of other drugs (eg, cisplatin); serum levels increase with cimetidine; magnesium trisilicate may decrease absorption |
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| Pregnancy | C - Safety for use during pregnancy has not been established.
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| Precautions | Caution in hepatic disease, G-6-PD deficiency, psoriasis, and porphyria; not recommended for long-term use in children; perform periodic (6 mo) ophthalmologic examinations; periodically test for muscle weakness; adverse effects are infrequent and include eye changes, GI symptoms (of which diarrhea is most prominent), and CNS changes |
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Drug Category: Corticosteroids
These agents are useful in adults or children with renal, CNS, or severe hematologic disease associated with SLE. Rarely needed in NLE but may be used in patients with severe hepatitis or thrombocytopenia.
| Drug Name | Prednisone (Deltasone, Orasone) |
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| Description | May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. |
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| Adult Dose | 5-60 mg/d PO qd or divided bid/qid |
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| Pediatric Dose | 1-2 mg/kg PO divided bid/qid |
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| Contraindications | Documented hypersensitivity; viral infection; peptic ulcer disease; hepatic dysfunction; connective tissue infections; fungal or tubercular skin infections; GI disease |
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| Interactions | Coadministration with estrogens may decrease prednisone clearance; concurrent use with digoxin may cause digitalis toxicity secondary to hypokalemia; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics |
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| Pregnancy | B - Usually safe but benefits must outweigh the risks.
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| Precautions | Abrupt discontinuation of glucocorticoids may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, cataract formation, growth suppression, and infections may develop with glucocorticoid use |
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Drug Category: Non-steroid topical immunosuppressants
These agents may be useful in postpubertal children with cutaneous SLE. Prolonged use of topical corticosteroids may produce significant skin adnexal atrophy.
| Drug Name | Pimecrolimus (Elidel) |
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| Description | Topical calcineurin inhibitor derived from ascomycin, a natural substance produced by the fungus Streptomyces hygroscopicus var ascomyceticus. Penetrates inflamed epidermis to inhibit T-cell activation by blocking transcription of proinflammatory cytokine genes such as interleukin-2, interferon gamma (Th1-type), interleukin-4, and interleukin-10 (Th2-type). Blocks catalytic function of calcineurin. Prevents release of inflammatory cytokines and mediators from mast cells in vitro after stimulation by antigen/IgE. Selectively inhibits production and release of inflammatory cytokines from activated T cells by binding to cytosolic immunophilin receptor macrophilin-12. The resulting complex inhibits phosphatase calcineurin, thus blocking T-cell activation and cytokine release. Cutaneous atrophy was not observed in clinical trials, which is a potential advantage over topical corticosteroids. |
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| Adult Dose | Apply topically to affected areas bid
Short-term and intermittent use only |
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| Pediatric Dose | <2 years: Not established >2 years: Administer bid as in adults Short-term and intermittent use only |
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| Contraindications | Documented hypersensitivity |
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| Interactions | CYP3A inhibitors may increase pimecrolimus levels in patients in whom increased absorption expected |
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| Pregnancy | C - Safety for use during pregnancy has not been established.
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| Precautions | Potential exacerbation of existing infection at site of application; may cause burning and irritation; caution with conditions that suppress the immune system (eg, AIDS, cancer); possible risk of lymph node or skin cancer based on animal studies and a small number of patients; may increase risk of viral infections (tacrolimus therapy has been associated with risk of developing eczema herpeticum, varicella zoster, and herpes simplex); other adverse effects include headache, sore throat, flulike symptoms, fever, and cough |
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| Drug Name | Tacrolimus (Protopic) |
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| Description | Topical calcineurin inhibitor - Penetrates inflamed epidermis to inhibit T-cell activation by blocking transcription of proinflammatory cytokine genes such as interleukin-2, interferon gamma (Th1-type), interleukin-4, and interleukin-10 (Th2-type). Blocks catalytic function of calcineurin. Prevents release of inflammatory cytokines and mediators from mast cells in vitro after stimulation by antigen/IgE. Also inhibits transcription for genes that encode IL-3, IL-4, IL-5, GM-CSF, and TNF-alpha, all of which are involved in the early stages of T-cell activation. Cutaneous atrophy was not observed in clinical trials, which is a potential advantage over topical corticosteroids. |
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| Adult Dose | Apply thin layer of ointment (concentrations of 0.03 and 0.1%) to affected skin areas bid and rub in gently and completely; continue treatment for 1 wk after clearing of signs and symptoms
Short-term and intermittent use only |
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| Pediatric Dose | <2 years: Not established
2-15 years: Apply 0.03% ointment bid to affected area(s)
>15 years: Administer as adults
Short-term and intermittent use only |
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| Contraindications | Documented hypersensitivity to tacrolimus or components of ointment |
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| Interactions | CYP3A inhibitors may increase levels in patients in whom increased absorption expected |
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| Pregnancy | C - Safety for use during pregnancy has not been established.
|
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| Precautions | Patients may experience a burning sensation during first few days of application; skin can become photosensitive and patients should be cautioned about exposure to direct or artificial sunlight and to use sunscreen; safety and efficacy in infected atopic dermatitis is not known; application under occlusion, which may promote systemic exposure, has not been evaluated (do not use tacrolimus ointment with occlusive dressings); absorption of tacrolimus following topical applications of tacrolimus ointment is minimal (relative to systemic administration), but tacrolimus is excreted in human milk, and, thus, a decision should be made whether to discontinue nursing or to discontinue drug, taking into account importance of drug to mother (potential for serious adverse reactions in nursing infants from tacrolimus should also be a concern); potential exacerbation of existing infection at site of application; may cause burning and irritation; caution with conditions that suppress the immune system (eg, AIDS, cancer); possible risk of lymph node or skin cancer based on animal studies and a small number of patients; may increase risk of viral infections (tacrolimus therapy has been associated with risk of developing eczema herpeticum, varicella zoster, and herpes simplex); other adverse effects include headache, sore throat, flulike symptoms, fever, and cough |
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Further Inpatient Care
- Inpatient care is not needed for patients with skin disease.
- Consider hospitalization and treatment with intravenous corticosteroids for patients with severe hematologic disease or CNS or renal involvement associated with SLE.
Further Outpatient Care
- Patients with neonatal lupus erythematosus (NLE) of the skin do not require monitoring after lesions resolve. They may be more prone to develop LE later in life, but this reflects their genetic predisposition, not that they had NLE. Their nonaffected siblings are also at risk for development of SLE.
- Patients with NLE with cardiac involvement require regular monitoring to assess cardiac function and the need for a pacemaker.
- SLE in children is often a chronic disease and requires regular evaluation and therapy modification.
Transfer
- Consider transfer to a tertiary care center for all children and neonates with LE.
Deterrence/Prevention
- Subsequent pregnancies
- Mothers of neonates with NLE, particularly neonates with CHB, have a 2- to 3-fold increased risk of subsequent affected neonates. An estimated 25% of subsequent pregnancies are affected. Therefore, carefully monitor subsequent pregnancies, particularly at 18-24 weeks’ gestation.
- 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, CHB was prevented with the use of corticosteroids beginning at 10 weeks’ gestation, azathioprine shortly thereafter, and plasmapheresis beginning at 18 weeks’ gestation.
Prognosis
- Neonatal lupus erythematosus
- Generally, skin disease is self-limited.
- Hepatosplenomegaly is self-limited, although death due to hepatitis may occur.
- Cytopenia is self-limited, but, if severe thrombocytopenia is present, bleeding can affect the prognosis.
- Cardiac involvement is associated with a poor outcome in many patients, including some who receive pacemakers.
- LE in childhood: Prognosis of children with SLE depends on the organ system affected.
Patient Education
- Assess and educate the mother about follow-up care for a child with NLE and the potential effects on subsequent pregnancies.
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- Instruct patients about sun avoidance, use of protective clothing, and proper use of sunscreens and topical corticosteroids.
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- Provide proper monitoring of patients while they are taking systemic corticosteroids or immunosuppressive agents.
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- For excellent patient education resources, visit eMedicine's Blood and Lymphatic System Center. Also, see eMedicine's patient education article Lupus (Systemic Lupus Erythematosus).
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- In mothers who are asymptomatic at the time of delivery of a neonate with cutaneous NLE or CHB, assess for the future development of LE or another collagen vascular disease.
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Medical/Legal Pitfalls
- Failure to diagnose
- Failure to recognize cardiac complications of neonatal lupus erythematosus (NLE)
- Failure to monitor subsequent pregnancies or the course of the mother
Special Concerns
- Prenatal ultrasonography may help identify NLE that affects the heart.
| Media file 1:
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. |
 |  View Full Size Image | |
Media type: Photo
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| Media file 2:
This child was one fraternal twin. Her sibling was not affected, although the mother and both infants had similar autoantibodies in their circulation. Eventually, the lesions resolved and healed without sequelae. |
 | View Full Size Image | |
Media type: Photo
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Neonatal Lupus and Cutaneous Lupus Erythematosus in Children excerpt Article Last Updated: Apr 4, 2007
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