Systemic lupus erythematosus in Child
Systemic lupus erythematosus (SLE) is a disease of unclear etiology characterized by immune abnormalities and multisystem involvement. Approximately 15%-20% of patients with SLE will present during the first 2 decades of life. Constitutional symptoms, fever, fatigue, weight loss, headache, mood disturbances, arthralgias, and skin findings may all be seen. Neurologic and renal involvement is prominent in childhood SLE. 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 in adolescence, but the incidence nearly equalizes in younger ages.
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.
There are drug-induced forms of the disease with a differing pattern of auto-immunity and clinical profile (discussed separately as drug-induced SLE).
Related topics: Oral lupus erythematosus, Subacute cutaneous lupus erythematosus, Discoid lupus erythematosus, Tumid lupus erythematosus
M32.9 – Systemic lupus erythematosus, unspecified
55464009 – Systemic lupus erythematosus
- Drug-induced SLE
- Dermatomyositis – Characteristic heliotrope rash (violaceous plaques surrounding eyes), photodistributed cutaneous eruption, and nailfold changes. Look for elevated serum CK levels and proximal symmetric extremity weakness.
- Stevens-Johnson syndrome – Characteristic target lesions, prominent systemic symptoms, but ANA and DIF negative.
- Antiphospholipid antibody syndrome / lupus anticoagulant – Can overlap with SLE; associated with recurrent thromboses and spontaneous abortions, elevated PT time.
- 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 – Without 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)