This summary discusses systemic lupus erythematosus in children. Neonatal lupus erythematosus is addressed separately.
Systemic lupus erythematosus (SLE) is a disease of unclear etiology characterized by immune abnormalities and multiorgan involvement. Approximately 15%-20% of patients with SLE will present during the first 2 decades of life. Childhood-onset SLE is a phenotype of SLE that presents in childhood or adolescence. It is relatively rare, with a worldwide prevalence estimated at 1.89-25.7 per 100 000 children. On average, it presents with a more aggressive phenotype than adult-onset SLE. Constitutional symptoms, fever, fatigue, anemia, weight loss, headache, mood disturbances, arthralgias, and skin findings may all be seen. Neurologic and renal involvement is a prominent characteristic in childhood SLE and often portends greater disease morbidity. Childhood SLE is also associated with chronic cardiopulmonary disease and macrophage activation syndrome.
The onset of the disease is usually between the ages of 10 and 15; however, it can occur at any age. Girls outnumber boys 8:1, but the ratio drops to 4:1 in adolescent cases. The disease is more common in people of color. It is found worldwide. Genetic factors play a role, and a family history of SLE or lupus erythematosus or an inherited complement deficiency in any form is a risk factor for developing the disease.
Childhood SLE is a chronic, noncurable disease that is treated with a variety of medications that impact immune function.
There are drug-induced forms of the disease with a differing pattern of autoimmunity and clinical profile.
Related topics: bullous systemic lupus erythematosus, discoid lupus erythematosus, oral lupus erythematosus, subacute cutaneous lupus erythematosus, tumid lupus erythematosus, lupus panniculitis
Systemic lupus erythematosus in Child
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Synopsis
Codes
ICD10CM:
M32.9 – Systemic lupus erythematosus, unspecified
SNOMEDCT:
55464009 – Systemic lupus erythematosus
M32.9 – Systemic lupus erythematosus, unspecified
SNOMEDCT:
55464009 – Systemic lupus erythematosus
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Differential Diagnosis & Pitfalls
- Malignancies – Acute lymphoblastic leukemia can be occasionally antinuclear antibody (ANA) positive.
- Infectious etiologies – Viral infections can at times result in positive ANA testing.
- Drug-induced SLE
- Dermatomyositis – Characteristic heliotrope rash (violaceous plaques surrounding eyes), photodistributed cutaneous eruption, and nail fold changes. Look for elevated serum creatinine kinase (CK) levels and proximal symmetric extremity weakness.
- Stevens-Johnson syndrome – Characteristic target lesions, prominent systemic symptoms, but ANA and direct immunofluorescent test (DIF) negative.
- Antiphospholipid antibody syndrome / lupus anticoagulant – Can overlap with SLE; associated with recurrent thromboses and spontaneous abortions, elevated prothrombin time (PT).
- Polymorphous light eruption (PMLE) – Most lesions resolve within several days; skin lesions are located primarily on sun-exposed areas (SLE can occur on sun-exposed and sun-protected areas). Note that previous studies have shown that up to 19% of patients with PMLE can be ANA positive. Hence, an ANA alone may not be sufficient in differentiating PMLE from SLE.
- Phototoxic / photoallergic drug eruptions
- CREST syndrome – Can have overlap with dermatomyositis. Refers to a subset of patients with limited scleroderma.
- Seborrheic dermatitis – No systemic findings. Erythema and scale in sebaceous distribution.
- Systemic amyloidosis
- Contact dermatitis
- Pityriasis rubra pilaris
- Scleroderma – Check for anticentromere antibodies and anti-Scl-70 antibodies. Typified by sclerotic changes in skin not seen in dermatomyositis.
- Graft-versus-host disease – Occurs after allogeneic stem-cell transplantation
- Generalized morphea – Asymmetric induration, no Raynaud phenomenon, no systemic involvement.
- Polymyositis – No cutaneous findings.
- Acute lesions of erythropoietic protoporphyria may have similar locations, especially on the dorsum of the hands, but usually there is no weakness.
- Tinea faciei – Check potassium hydroxide (KOH); will also be ANA negative.
- Chilblains (perniosis)
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Last Reviewed:02/07/2019
Last Updated:01/11/2021
Last Updated:01/11/2021
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Systemic lupus erythematosus in Child
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