What are the symptoms, causes of lupus disease? The diagnosis and treatment of Lupus.
Lupus; is a name applied to two unrelated diseases: lupus erythematosus (LE) and lupus vulgaris.
There are two forms of lupus erythematosus: a skin form called discoid LE and an internal form called systemic LE, or SLE. They afflict several hundred thousand people in the United States alone, with SLE making up about 50% of the cases. SLE affects women nine times as often as men, most commonly between the ages of 20 and 40.
Cause: The cause of lupus erythematosus is unknown, but it is suspected that an irregularity in the body’s immune mechanism may be involved. It is known that the blood of most patients contains abnormal antibodies against the nucleoprotein DNA. These antibodies cause some of the body’s leukocytes (white blood cells) to engulf large masses of DNA from worn-out body cells. These leukocytes are called LE cells. In addition to the anti-DNA antibodies, LE patients may also have antibodies against other body cells, including red blood cells, white blood cells, and platelets.
Although it is not known what causes the formation of the abnormal antibodies, certain drugs have been found to cause similar changes in the blood. The most widely used of these drugs is procaine amide (Pronestyl), used in the treatment of heart irregularities. It has been suggested that the rising incidence of SLE is related to increased use of drugs and food additives, exposure to pollutants, and other factors.
Symptoms: Although there are two forms of LE, there may be considerable overlap in their symptoms, and about 10% of the patients with discoid LE eventually develop SLE. The skin lesions in both forms tend to occur on areas exposed to light, and sometimes they are identical. In discoid lupus, red, slightly scaly lumps appear, usually on the face. If untreated the spots become scars. In typical systemic lupus there is a generalized redness on the cheeks and across the bridge of the nose, an area called the butterfly zone because of its resemblance to the shape of a butterfly. Usually, there is little scarring.
Patients with discoid LE occasionally have minor aching in the joints, particularly those of the hands. This same symptom occurs in about 90% of the patients with SLE. In fact, SLE most often begins as a form of arthritis, and the skin lesions and other symptoms appear later. About 80% of the patients have fever, 70% have some skin changes, 60% have lymph node enlargement, 55% have anemia, 50% have unexplained nausea, vomiting, and diarrhea, and 40% have a low white blood count. About half of all SLE patients suffer generalized muscle aching and kidney damage. The course of SLE is characterized by periods of spontaneous remission and frequent flareups. Infections and excessive exposure to sunlight may increase the severity of the symptoms.
Diagnosis: The diagnosis of discoid LE is usually based on the typical appearance of the skin lesions. Sometimes a biopsy is performed. A diagnosis of systemic LE is often made on the basis of the patient’s symptoms and can usually be confirmed by the finding of LE cells or abnormal antibodies in the patient’s blood.
Treatment: The treatment of the skin lesions of both discoid and systemic lupus is similar. If the patient is sensitive to sunlight, he should avoid excessive exposure to sun and should use lotions and other substances that block out the sun’s rays. Small lesions can be controlled by the topical application of medications containing cortisone derivatives. Larger lesions can be treated with antimalarial drugs such as hydroxychloroquine (Plaquenil), chloroquine (Aralen), or quinacrine (Atabrine).
The treatment of systemic LE depends largely on the manifestations of the disease. For example, mild aching in the joints may be treated with large doses of aspirin, and severe aching with drugs normally used for rheumatoid arthritis. Immunosuppressive drugs have been used only on an experimental basis.
Prognosis: The prognosis of discoid LE is excellent if treatment is started early. Some patients may require long-term antimalarial therapy to prevent extension of their skin lesions. The outlook for SLE patients is not so good but has improved markedly. Prior to the discovery of the anti-inflamatory action of cortisone in 1949, half the patients died within two years of their first symptoms, but half of them now survive more than six years. As research on LE continues, the life-span will undoubtedly continue to increase.
Lupus vulgaris is actually tuberculosis of the skin. It is a very rare disease in which pimplelike lesions slowly spread over larger areas. There is always scar formation, and the skin may become permanently mutilated. Treatment is usually the same as for other forms of tuberculosis—chiefly the administration of such drugs as isonicotinic acid hydrazide. Small skin lesions are sometimes removed surgically.
***This article is for informational purposes only. It is not a doctor warning or recommendation.