Systemic lupus erythematosus in Child
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Synopsis
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
Codes
ICD10CM: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
Patient Information for Systemic lupus erythematosus in Child
Overview
Systemic lupus erythematosus (SLE), also called lupus, is a chronic inflammatory disease that can affect almost any part of the body, especially the skin, joints, kidneys, heart, lungs, bones, blood, or brain. Systemic lupus erythematosus is considered an autoimmune disorder, meaning that a person's own immune system attacks his or her own healthy cells and tissues, causing inflammation and damage.Because systemic lupus erythematosus can affect any organ system, no two people have identical forms of the disease. However, most people with systemic lupus erythematosus report periods of time in which their symptoms seem to be mild or absent (remission) and other periods of time when the inflammation is more severe (flare or relapse).
Who’s At Risk
Systemic lupus erythematosus can occur in people of all ages, all races, and both sexes. However, it is far more common in women, especially those between 15-45 years old. In America, it is also more commonly seen in people with darker skin than in light-skinned people.Although it is not directly inherited, lupus and other autoimmune diseases may run in families. Inheriting certain genes may make some people more susceptible to developing lupus.
In addition, certain environmental factors may trigger lupus in those who have a family (genetic) tendency toward the disease, including:
- Ultraviolet light, especially sunlight
- Certain medications, especially hydralazine and procainamide
- Infections
- Antibiotics, especially penicillins or sulfa-containing medicines
- Stress
- Hormonal changes, especially related to pregnancy and menstrual cycles
Signs & Symptoms
More than 90% of people with systemic lupus erythematosus have skin symptoms. The most common locations for the skin lesions of systemic lupus erythematosus include:- Face, especially cheeks and nose
- Sun-exposed skin on arms, backs of hands, upper chest, and upper back due to increased sensitivity to sunlight (photosensitivity)
- Fingers and fingernails
- Mouth or nose
- Scalp
A rash can develop in sun-exposed skin (photo-distribution), especially on the backs of the hands and fingers. This rash, which appears as red, scaly patches, can also affect the arms and trunk.
The skin around fingernails (nail folds) can be red and inflamed, and tiny, dilated blood vessels (telangiectasia) may be seen. In addition, people may develop Raynaud phenomenon, in which the fingers (and sometimes toes) turn pale and numb after exposure to cold temperatures.
Small, painless ulcers can develop in the nose or, more commonly, in the mouth, especially on the roof of the mouth.
When lupus affects the scalp skin, you may notice hair loss. It may be patchy, or there may be thinning across the scalp, especially at the temples.
In addition to the skin lesions of lupus, people may have:
- Joint pain or swelling, especially in hands, wrists, and knees
- Blood problems, including anemia and clotting disorders
- Kidney disorders
- Lung problems, such as painful breathing
- Seizures or other brain disorders
- Swollen lymph glands
- Fever
- Fatigue
Self-Care Guidelines
If you know you have systemic lupus erythematosus, several measures can help prevent flares:- Avoid intense sun exposure.
- Apply sunscreen with SPF of 30 or higher every day.
- Maintain healthy habits such as resting well, eating a balanced diet, and exercising regularly.
- Reduce stress.
- Avoid smoking and limit alcohol use.
When to Seek Medical Care
See your doctor if you develop a rash with fever and fatigue.Treatments
Lupus can be difficult to diagnose for 3 reasons: systemic lupus erythematosus can affect so many different organ systems, its symptoms can come and go, and no 2 people have exactly the same form of the disease. In addition to a careful review of your medical history, your doctor may perform blood tests, urinalysis, chest X-ray, or an electrocardiogram (ECG) before confirming the diagnosis of lupus.If you have a rash that is suspicious for lupus, you nay need a skin biopsy. The procedure involves:
- Numbing the skin with an injectable anesthetic.
- Sampling a small piece of skin by using a flexible razor blade, a scalpel, or a tiny cookie cutter (called a "punch biopsy"). If a punch biopsy is taken, a suture or two may be placed and will need to be removed 6-14 days later.
- Having the skin sample examined under the microscope by a specially trained physician (dermatopathologist).
- Anti-malarial drugs such as hydroxychloroquine, chloroquine, or quinacrine
- Corticosteroids
- Anti-inflammatory medications such as aspirin, ibuprofen, naproxen, or indomethacin
- Immune-suppressing medications including azathioprine, cyclophosphamide, methotrexate, cyclosporine, chlorambucil, or mycophenolate mofetil
References
Bolognia, Jean L., ed. Dermatology, pp.594-595. New York: Mosby, 2003.
Freedberg, Irwin M., ed. Fitzpatrick's Dermatology in General Medicine. 6th ed, pp.1619, 1677, 1681-1687. New York: McGraw-Hill, 2003.
Systemic lupus erythematosus in Child
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