Lupus Erythematosus

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lupus erythematosus

[′lü·pəs ‚er·ə‚thē·mə′tō·səs]
(medicine)
An acute or subacute febrile collagen disease characterized by a butterfly-shaped rash over the cheeks and perilingual erythema.

Lupus Erythematosus

 

a disease occurring in two distinct forms: (1) discoid lupus erythematosus, a chronic disease of the skin that usually does not alter the patient’s general condition (the disease was described in detail in 1851 by the French physician P. Cazenave), and (2) systemic lupus erythematosus (first identified under the name acute lupus erythematosus in 1872 by the Austrian physician M. Kaposi), a serious disease affecting many internal organs with systemic involvement of connective tissue and blood vessels. It belongs to the group classified as collagen diseases. Both forms are related inasmuch as discoid lupus erythematosus occasionally progresses into the systemic form. The etiology of the disease has not been conclusively determined.

Discoid lupus erythematosus is manifested by skin lesions in the form of red patches covered with whitish-gray scales. These patches gradually enlarge, and the skin in the center soon thins and a small scar forms. It occurs most frequently on the face in a symmetrical fashion, extending from the bridge of the nose to the cheeks in a butterfly-like shape. The auricles, scalp, chest, and, less commonly, the extremities are also affected. The disease persists for years and decades, worsening in the spring and summer.

Systemic lupus erythematosus is characterized by great variation of symptoms from patient to patient. All patients suffer from joint problems, such as intermittent or persistent pain, swelling and reddening, and, less commonly, deformation of the small joints of the hands. The muscles and tendons are also likely to be involved, and peculiar transient contractures appear. A variety of skin rashes is often encountered. The lesion on the bridge of the nose spreading to the cheeks, the butterfly-like rash, is characteristic. Specific lesions may develop on the mucous membranes of the oral cavity and on the lips. Many patients suffer loss of hair, dry skin, and brittle nails. Involvement of the serous membranes is a part of the characteristic triad of dermatitis, polyarthritis, and polyserositis. Lupus erythematosus may also cause myocarditis and endocarditis, various inflammatory processes of the lung (pneumonitis), nephritis, and lesions of the nervous system. (At the beginning there may be irritability or depression, and insomnia; later encephalitis, myelitis, or polyneuritis develop.) Sometimes the gastrointestinal system is involved, including liver and spleen enlargement. The lymphatic system may be impaired by the disease. Anemia, leukopenia, and thrombocytopenia develop. The eyes are affected. A typical laboratory finding of lupus erythematosus is the presence of special cells (the so-called LE cells) and antinuclear antibodies in the blood. The course of systemic lupus erythematosus may be acute, subacute, or chronic, depending on the severity of the initial phase and the general course of the disease.

Treatment includes long-term administration of synthetic antimalarial agents (Delagil, chloroquinum, Resochin), which have an anti-inflammatory and desensitizing effect, and vitamins B2, B12, and nicotinic acid. Discoid lupus erythematosus is treated by gold compounds and local application of ointments (prednisolone, Locacorten, Synalar, and zinc ointment for protection against the sun). Systemic lupus erythematosus is treated by a combination of cortico-sterone hormones (prednisolone, triamcinolone, dexamethasone) and antimalarial agents and antibiotics (to treat or prevent infection) and by general supportive measures.

REFERENCES

Arutiunov, V. Ia. Krasnaia volchanka. Moscow, 1961. (Bibliography.)
Nesterov, A. I., and Ia. A. Sigidin. Klinika kollagenovykh boleznei, 2nd ed. Moscow, 1966. (Bibliography.)
Tareev, E. M. Kollagenozy. Moscow, 1965. (Bibliography.)

V. A. NASONOVA

References in periodicals archive ?
Histologic findings in cutaneous lupus erythematosus. Arch Dermatol 1994; 130: 54-8.
Cutaneous lupus erythematosus: An update on pathogenesis, diagnosis, and treatment.
Furthermore, iNKT cells which also express perforin, granzyme B, and Fas L are believed to play a role in the pathogenesis of cutaneous lupus erythematosus [46, 47].
Prevalence of self-report photosensitivity in cutaneous lupus erythematosus. J Am Acad Dermatol 2012;66(2):2208.
Cutaneous lupus erythematosus: updates on pathogenesis and associations with systemic lupus.
Cutaneous lupus erythematosus is characterized by local activation of immune complexes or complement, autoreactive B cells and T cells and overexpression of cytokines and chemokines [108].
The CLASI (Cutaneous Lupus Erythematosus Disease Area and Severity Index): An Outcome Instrument for Cutaneous Lupus Erythematosus.
- Chronic Cutaneous Lupus Erythematosus (CCLE) / Discoid Lupus Erythematosus (DLE) Global Clinical Trials Review, H2, 2014
Nyberg, "Cutaneous lupus erythematosus and the association with systemic lupus erythematosus: a population-based cohort of 1088 patients in Sweden," The British Journal of Dermatology, vol.
Cutaneous lupus erythematosus without systemic lupus erythematosus: clinical and laboratory features.
Multiple, keratoacanthoma-like nodules on a 47-year-old man: a rare presentation of cutaneous lupus erythematosus. Int J Dermatol.
* Cutaneous lupus erythematosus, which includes discoid lupus (DLE), involves the development of lesions on the face or other sun-exposed areas.

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