cutaneous lupus erythematosus


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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.

cu·ta·ne·ous lu·pus er·y·the·ma·to·sus

1. skin disease seen in patients with discoid form of lupus erythematosus;
2. a term for various skin lesions seen in systemic lupus erythematosus.

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.

cu·ta·ne·ous lu·pus er·y·the·ma·to·sus

(kyū-tānē-ŭs lūpŭs eri-thē-mă-tōsis)
1. Skin disease seen in patients with discoid form of lupus erythematosus
2. Various skin lesions seen in systemic lupus erythematosus
References in periodicals archive ?
Plasmacytoid dendritic cells: an overview of their presence and distribution in different inflammatory skin diseases, with special emphasis on Jessner's lymphocytic infiltrate of the skin and cutaneous lupus erythematosus.
Familial chilblain lupus, a monogenic form of cutaneous lupus erythematosus, maps to chromosome 3p.
A snapshot of the global therapeutic scenario for Chronic Cutaneous Lupus Erythematosus (CCLE) / Discoid Lupus Erythematosus (DLE).
Prompt recognition of drug-induced subacute cutaneous lupus erythematosus (SCLE) by physicians prevents significant morbidity, both by quelling the number and activity of skin lesions and by sparing affected patients from exposure to the potent immunosuppressive agents that are often used in idiopathic SCLE.
A snapshot of the global therapeutic scenario for Cutaneous Lupus Erythematosus.
During her presentation at the meeting of the European Society of Cutaneous Lupus Erythematosus, which was held in conjunction with the EADV congress, audience members noted that there has been a dearth of population-based studies reporting the incidence of isolated CLE.
The Company currently has three development projects in Phase II clinical studies for the treatment of hand dermatitis, seborrheic dermatitis and cutaneous lupus erythematosus in addition to a broad portfolio of product candidates and research programs in earlier phases.
Finally, etanercept therapy successfully treated refractory dermatomyositis in a patient with a history of breast cancer and was also used successfully to treat subacute cutaneous lupus erythematosus with prevention of cutaneous eruption, according to two posters presented at the meeting by J.
TolerRx has completed a Phase I trial of TRX1 and is currently enrolling subjects with refractory cutaneous lupus erythematosus to participate in a Phase Ib study.
The present Competitive Intelligence Report about new developments in the pipeline for treatment of Systemic Lupus Erythematosus (SLE), Lupus Nephritis and Cutaneous Lupus Erythematosus (CLE) provides a competitor evaluation in the field of R&D projects for treatment of SLE, Lupus Nephritis and CLE as of February 2008.
TolerRx has two antibodies in clinical development: TRX4 in type 1 diabetes and psoriasis, and TRX1 in cutaneous lupus erythematosus (CLE).
The investigators found that the skin lesions of systemic lupus erythematosus (SLE) and subacute cutaneous lupus erythematosus (LE) responded better than those of long-standing discoid LE, which tend to be more hyperkeratotic and therefore resistant to transdermal drug penetration.

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