DLE


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lupus

 [loo´pus]
a name originally given to a destructive type of skin lesion, implying a local degeneration rather than systemic involvement. Although the term is frequently used alone to designate lupus vulgaris and sometimes lupus erythematosus, without a modifier it has no meaning. The Latin word lupus means wolf; erythematosus refers to redness. The name lupus erythematosus has been used since the 13th century because physicians thought the shape and color of the skin lesions resembled the bite of a wolf. Currently, there are at least two recognized manifestations of the disease: discoid lupus erythematosus and systemic lupus erythematosus.
chilblain lupus erythematosus a form of discoid lupus erythematosus aggravated by cold, initially resembling chilblains, in which the lesions consist of reddened infiltrated patches on the exposed areas of the body, especially the finger knuckles.
cutaneous lupus erythematosus one of the two main types of lupus erythematosus; it may involve only the skin or may precede involvement of other body systems. It may be chronic (discoid lupus erythematosus); subacute (systemic lupus erythematosus); or acute (characterized by an acute edematous, erythematous eruption, often with systemic exacerbations). The acute form may be the presenting symptom of systemic lupus erythematosus, such as after sun exposure.
discoid lupus erythematosus (DLE) a superficial inflammation of the skin, marked by red macules up to 3 to 4 cm in width, and covered with scanty adherent scales, which extend into spreading follicles that fall off and leave scars. The lesions typically form a butterfly pattern over the bridge of the nose and cheeks, but other areas may be involved, notably the scalp and other areas that are exposed to light.
drug-induced lupus a syndrome closely resembling systemic lupus erythematosus, precipitated by prolonged use of certain drugs, most commonly hydralazine, isoniazid, various anticonvulsants, and procainamide.
lupus erythemato´sus (LE) a group of connective tissue disorders primarily affecting women aged 20 to 40, comprising a spectrum of clinical forms in which cutaneous disease may occur with or without systemic involvement.
lupus per´nio
1. soft, purplish skin lesions on the cheeks, forehead, nose, ears, and digits, frequently associated with bone cysts, which may be the first manifestation of sarcoidosis or occur in the chronic stage of the disease.
systemic lupus erythematosus (SLE) a chronic inflammatory disease, usually febrile and characterized by injury to the skin, joints, kidneys, nervous system, and mucous membranes. It can, however, affect any organ of the body and usually has periods of remissions and exacerbations. (See plate in Dermatology Atlas.)

It was once thought that this was a fairly rare disease, but improved immunologic testing procedures have shown that it is not. It is primarily a disease of women, occurring five to ten times more often in females than in males. Although the peak incidence is between 30 and 40 years of age, the condition has also been diagnosed in the very young and the very old.

SLE is the classic prototype of autoimmune disease of connective tissue. Its etiology is unknown, but the high level of autoantibodies in persons with the condition indicates a defect in the regulatory mechanisms that sustain self-tolerance and prevent the body from attacking its own cells, cell constituents, and proteins. Patients with SLE can have a wide variety of autoantibodies against nuclear and cytoplasmic cellular components. The presence of high levels of antinuclear antibody (ANA) in SLE patients with glomerulonephritis indicates a pathogenic role for that antibody. The antibodies are directed against deoxyribonucleoprotein, DNA, histone, and a soluble non–nucleic acid molecule called Sm antigen.

Factors that appear to contribute to the development of SLE include exposure to sunlight or ultraviolet radiation from sunlamps, a genetic predisposition to the disease, certain drugs, viral infections, and hormonal influences.

Clinical manifestations of SLE are confusingly diverse owing to the involvement of connective tissue throughout the body. Typically, the patient seeks medical help for relief of fever, weight loss, joint pain, the characteristic butterfly rash, pleural effusion and pleuritic pain, and nephritis. The detection of ANA by microscopic immunofluorescence is supportive evidence for the presence of SLE.

Either glomerulonephritis, which is usually mild, or cardiovascular manifestations such as myocarditis, endocarditis, or pericarditis, are found in about half the patients with SLE. Pulmonary disease, especially pleurisy, is also relatively common, as are gastrointestinal disturbances and lymph node involvement. Organic neurologic disturbances produce behavioral aberrations and frank psychosis in some patients; in a few others, there are peripheral neuropathies, motor weakness, and diplopia.

Supportive measures are used to prevent or minimize acute relapses and exacerbations of symptoms. The patient is instructed to avoid exposure to sunlight and ultraviolet radiation from other sources, blood transfusions, penicillin, and the sulfonamides. Active disease is treated with topical steroids, nonsteroidal antiinflammatory drugs for fever and joint pain, corticosteroids, and immunosuppressants. The goal of drug therapy is suppression of the immune system. Treatment of specific manifestations of SLE is aimed at prevention of complications. Physical therapy may be required to alleviate muscle weakness and prevent orthopedic deformities.
lupus vulga´ris the most common and severe form of tuberculosis of the skin, most often affecting the face, with formation of reddish brown patches of nodules in the corium, which progressively spread peripherally with central atrophy, causing ulceration and scarring and destruction of cartilage in involved sites.

DLE

discoid lupus erythematosus.

DLE

abbreviation for discoid lupus erythematosus.

lupus

(loo'pus) [L. lupus, wolf]
Originally any chronic, progressive, usually ulcerating, skin disease. In current usage, when the word is used alone, it has no precise meaning.

discoid lupus erythematosus

Abbreviation: DLE
A chronic skin disease characterized by periodic acute appearances of a scaling, red, macular rash. DLE is caused by an autoimmune process involving both B-cell– and T-cell–mediated mechanisms that destroy the skin's basal cells. DLE is treated with topical corticosteroids. It is found in about 5% to 30% of patients who have systemic lupus erythematosus (SLE) (esp. those who smoke) but also may occur alone (without other findings of SLE). See: autoimmune disease; systemic lupus erythematosus

Treatment

The patient should avoid exposure to the sun. Skin lesions should be treated with topical corticosteroids, but overuse of these preparations should be avoided.

drug-induced systemic lupus erythematosus

A group of signs and symptoms similar to those of systemic lupus erythematosus, caused by an adverse reaction to drugs, esp. procainamide, hydralazine, and isoniazid. Joint inflammation and pain, skin rash, pleurisy, and fever are the most common manifestations; kidney and central nervous system involvement are rare. Antinuclear antibodies, specifically against the histones that fold DNA, are common. Some patients develop antinuclear antibodies but do not develop lupus-like symptoms. The lupus-like syndrome usually disappears when the drug causing it is discontinued. See: antinuclear antibodies; systemic lupus erythematosus

lupus erythematosus

Any of several chronic, progressive, ulcerating, skin diseases, esp. systemic lupus erythematosus.

neonatal lupus

Rash, abnormally low platelet counts, liver and brain disease, and congenital heart block occurring in an infant whose mother has systemic lupus erythematosus. The disease results from the passage of maternal autoantibodies to the developing fetus. Although most of the findings resolve spontaneously, congenital heart block does not, and it may require the insertion of a pacemaker.

lupus panniculitis

Lupus profundus.

lupus pernio

Purple, noncaseating granulomas occurring on the face, esp. around the nose, eyes, cheeks, lips, and ears. Lupus in lupus pernio is misleading because it suggests a connection with systemic lupus erythematosus; lupus pernio is actually a finding of the skin in sarcoidosis.

lupus profundus

A deeply scarring, atrophic rash occasionally found in patients with systemic lupus erythematosus, caused by inflammation of subcutaneous fatty tissue.
Synonym: lupus panniculitis
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SYSTEMIC LUPUS ERYTHEMATOSUS

systemic lupus erythematosus

Abbreviation: SLE
A chronic autoimmune inflammatory disease of connective tissue involving multiple organ systems and marked by periodic acute episodes. Its name is derived from the characteristic erythematous butterfly rash over the nose and cheeks, which resembles a wolf's snout, although this is present in less than 50% of patients. The disease is most prevalent in women (ratio of 8:1 women:men) of childbearing age (ratio of 15:1). Although it occurs worldwide, it is most prevalent among black and Asian peoples.

Etiology and Pathology

SLE is classified as an autoimmune disease in which the body seems to be unable to maintain normal mechanisms of tolerance to autoantigens. Activation of T helper cells and B cells results in the production of autoantibodies that attack antigens in the cytoplasm and nucleus of cells and on the surface of blood cells. The exact cause of SLE is unknown: genetic defects, hormonal changes, infection, physical or mental stress, some drugs, immunizations, and environmental triggers (sunlight, UV light exposure) are possible predisposing factors. See: autoimmune disease; glomerulonephritis

Autoantibodies can react with autoantigens to form immune complexes in such large numbers that they cannot be completely excreted; the immune complexes may precipitate within blood vessels, producing inflammation at the site and disrupting the flow of blood and oxygen to tissues. These deposits are particularly damaging in the glomeruli. Autoantibodies also promote the destruction of cells by stimulating neutrophil and macrophage phagocytic activity, which increases cell destruction from trauma, infection, or drugs.

Diagnosis

In 1997, revised criteria for diagnosis of SLE were established. The diagnosis can be made if four or more of the following criteria are present, either at one time or sequentially: (1) butterfly rash; (2) raised, scaly discoid skin lesions; (3) abnormal titer of antinuclear antibodies seen by immunofluorescence; (4) other autoantibodies (anti-Sm; serological tests for syphilis); (5) pleuritis or pericarditis (together referred to as “serositis”); (6) hemolytic anemia, leukopenia (white blood cell count less than 4,000 mm3), lymphopenia (lymphocyte count less than 1,500/mm3), or thrombocytopenia of less than 100,000/mm3; (7) oral or nasopharyngeal ulcers; (8) nonerosive arthritis; (9) psychosis or seizures without other clear cause; (10) photosensitivity skin rash; and (11) proteinuria greater than 0.5 g/day or cellular casts in the urine.

Some drugs can cause a lupus-like syndrome; the most common of these are procainamide, isoniazid, and hydralazine. See: drug-induced systemic lupus erythematosus

Symptoms

The onset of the disease may be acute or insidious. Patients have a wide variety of clinical symptoms, signs, and laboratory findings, but anemia, thrombocytopenia, polyarthritis, (polyarthralgia) skin rashes, glomerulonephritis, fever, malaise, weight loss, fatigue, and low blood levels of complement are the most common. Other signs include pleuritis, pericarditis, myocarditis, neurological changes including behavioral changes and seizure activity (neural lupus), gastrointestinal ulcerations, Raynaud's phenomenon (present in about 20% of patients), and other problems caused by inflammatory changes of the blood vessels or connective tissue. Most patients are prone to infection.

Treatment

No cure for SLE exists, and complete remission is rare. About 25% of patients have mild disease, demonstrating only minor skin and hematological signs, and can be treated with nonsteroidal anti-inflammatory drugs for their arthritis symptoms and topical treatment (sometimes with corticosteroid creams) for skin lesions. Rashes may respond to antimalarials, e.g., hydroxychloroquine, but patients must be observed closely for the possibility of drug-induced retinal damage. Other treatments for skin rash include quinacrine, retinoids, and dapsone. Life-threatening and severely disabling conditions should be treated with high doses of corticosteroids and supplemental calcium to minimize osteoporosis, which may be an undesired side effect of long-term glucocorticoid use. Immunosuppressive drugs are used for severe exacerbations and to reduce steroid dosage.

Prognosis

The prognosis depends on which organ systems are involved, how severely they are damaged, and how rapidly the disease progresses. Ten-year survival rates are high (80%). Renal failure and infections are the most common causes of death.

Patient care

Patient education related to the disease, diagnostic procedures, and treatment is essential in lupus, as in any chronic disease. Ongoing assessment is carried out to assess flares of the illness. The purpose, proper dosage, use, and side effects of drugs is taught. Patients need emotional support to help cope with changes in appearance. Patients should be taught to wear clothing and hats that block direct sunlight, use a sunscreen with a 15 or higher protection factor, and to maintain a diet appropriate for their renal functional status. The health care professional should help establish a regimen for adequate relief of both the musculoskeletal pain and chronic fatigue experienced by most patients, encouraging adequate rest. Heat packs relieve joint stiffness and pain, and regular gentle exercise helps to maintain full range of motion. Physical and occupational therapy consultations are provided as appropriate. Additional support and teaching depend on the organ system most affected by the disease. If the female patient of childbearing age has no renal or neurologic impairment, she can have a safe, successful pregnancy if desired. Over time, patients with severe progressive disease need assistance in coping with chronic illness and the possibility of mortality. Referrals to the Lupus Foundation of America (202-349-1155; www.lupus.org) and the Arthritis Foundation (800-283-7800; www.arthritis.org) are helpful.

See: illustration

lupus vulgaris

Tuberculosis of the skin; characterized by patches that break down and ulcerate, leaving scars on healing.

discoid lupus erythematosus

Abbreviation: DLE
A chronic skin disease characterized by periodic acute appearances of a scaling, red, macular rash. DLE is caused by an autoimmune process involving both B-cell– and T-cell–mediated mechanisms that destroy the skin's basal cells. DLE is treated with topical corticosteroids. It is found in about 5% to 30% of patients who have systemic lupus erythematosus (SLE) (esp. those who smoke) but also may occur alone (without other findings of SLE). See: autoimmune disease; systemic lupus erythematosus

Treatment

The patient should avoid exposure to the sun. Skin lesions should be treated with topical corticosteroids, but overuse of these preparations should be avoided.

See also: lupus

DLE

discoid lupus erythematosus.
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