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Lupus erythematosus

Lupus erythematosus (also known as systemic lupus erythematosus or SLE) is an autoimmune disorder in which antibodies are created against the patient's own DNA. It can cause various symptoms, but the main ones relate to the skin, kidney (lupus nephritis), joints, blood and immune system. more...

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It is named for the Latin lupus, meaning "wolf", perhaps due to a crude similarity between the facial rash associated with the illness, and a wolf's face, although various explanations exist.

Signs and symptoms

Common initial and chronic complaints are fever, malaise, joint pains, myalgias and fatigue. Because they are so often seen with other diseases, these signs and symptoms are not part of the diagnostic criteria for SLE. When occurring in conjunction with other signs and symptoms, however, they are considered suggestive.

Dermatological manifestations

As many as 30% of patients present with some dermatological symptoms (and 65% suffer such symptoms at some point), with 30% to 50% suffering from the classic malar (or butterfly) rash associated with the disease. Patients may present with discoid lupus (thick, red scaly patches on the skin). Alopecia, mouth and vaginal ulcers, and lesions on the skin are also possible manifestations.

Musculoskeletal manifestations

Patients most often seek medical attention for joint pain, with small joints of the hand and wrist usually affected, although any joint is at risk. Unlike rheumatoid arthritis, SLE arthropathy is not usually destructive of bone, however, deformities caused by the disease may become irreversible in as many as 20% of patients.

Hematological manifestations

Anemia and iron deficiency may develop in as many as half of patients. Low platelet and white blood cell counts may be due to the disease or a side-effect of pharmacological treatment.

Cardiac manifestations

Patients may present with inflammation of various parts of the heart: pericarditis, myocarditis and endocarditis. The endocarditis of SLE is characteristically non-infective (Libman-Sacks endocarditis), and involves either the mitral valve or the tricuspid valve. Atherosclerosis also tends to occur more often and advance more rapidly in SLE patients than in the general population. (Asanuma et al 2003, Bevra 2003, Roman et al 2003).

Renal involvement

Painless hematuria or proteinuria may often be the only presenting renal symptom. Acute or chronic renal impairment may develop with lupus nephritis, leading to acute or end stage renal failure. Because of early recognition and management of SLE, end stage renal failure occurs in less than 5% of patients.

Neurological manifestations

About 10% of patients may present with seizures or psychosis. A third may test positive for abnormalities in the cerebrospinal fluid.

T-cell abnormalities

Abnormalities in T cell signaling are associated with SLE, including deficiency in CD45 phosphatase, increased expression of CD40 ligand. Also associated with SLE is increased expression of FcεRIγ, which replaces the TCR ζ chain, which is deficient in some SLE patients. Other abnormalities include:

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Discoid lupus erythematosus
From Gale Encyclopedia of Medicine, 4/6/01 by Rosalyn S. Carson-DeWitt

Definition

Discoid lupus erythematosus (DLE) is a disease in which coin-shaped (discoid) red bumps appear on the skin.

Description

The disease called discoid lupus erythematosus only affects the skin, although similar discoid skin lesions can occur in the serious disease called systemic lupus erythematosus (SLE). Only about 10% of all patients with DLE will go on to develop the multi-organ disease SLE.

The tendency to develop DLE seems to run in families. Although men or women of any age can develop DLE, it occurs in women three times more frequently than in men. The typical DLE patient is a woman in her 30s.

Causes & symptoms

The cause of DLE is unknown. It is thought that DLE (like SLE) may be an autoimmune disorder. Autoimmune disorders are those that occur when cells of the immune system are misdirected against the body. Normally, immune cells work to recognize and help destroy foreign invaders like bacteria, viruses, and fungi. In autoimmune disorders, these cells mistakenly recognize various tissues of the body as foreign invaders, and attack and destroy these tissues. In SLE, the misdirected immune cells are antibodies. In DLE, the damaging cells are believed to be a type of white blood cell called a T lymphocyte. The injury to the skin results in inflammation and the characteristic discoid lesions.

In DLE, the characteristic skin lesion is circular and raised. The reddish rash is about 5-10 mm in diameter, with the center often somewhat scaly and lighter in color than the darker outer ring. The surface of these lesions is sometimes described as "warty." There is rarely any itching or pain associated with discoid lesions. They tend to appear on the face, ears, neck, scalp, chest, back, and arms. As DLE lesions heal, they leave thickened, scarred areas of skin. When the scalp is severely affected, there may be associated hair loss (alopecia).

People with DLE tend to be quite sensitive to the sun. They are more likely to get a sunburn, and the sun is likely to worsen their discoid lesions.

Diagnosis

Diagnosis of DLE usually requires a skin biopsy. A small sample of a discoid lesion is removed, specially prepared, and examined under a microscope. Usually, the lesion has certain microscopic characteristics that allow it to be identified as a DLE lesion. Blood tests will not reveal the type of antibodies present in SLE, and physical examination usually does not reveal anything other than the skin lesions. If antibodies exist in the blood, or if other symptoms or physical signs are found, it is possible that the discoid lesions are a sign of SLE rather than DLE.

Treatment

Treatment of DLE primarily involves the use of a variety of skin creams. Sunscreens are used for protection. Steroid creams can be applied to decrease inflammation. Occasionally, small amounts of a steroid preparation will be injected with a needle into a specific lesion. Because of their long list of side effects, steroid preparations taken by mouth are avoided. Sometimes, short-term treatment with oral steroids will be used for particularly severe DLE outbreaks. Medications used to treat the infectious disease malaria are often used to treat DLE.

Alternative treatment

Alternative treatments for DLE include eating a healthy diet, low in red meat and dairy products and high in fish containing omega-3 fatty acids. These types of fish include mackerel, sardines, and salmon. Following a healthy diet is thought to decrease inflammation. Dietary supplements believed to be helpful include vitamins B, C, E, and selenium. Vitamin A is also recommended to improve DLE lesions. Constitutional homeopathic treatment can help heal DLE as well as help prevent it developing into SLE.

Prognosis

For the most part, the prognosis for people with DLE is excellent. While the lesions may be cosmetically unsightly, they are not life threatening and usually do not cause a patient to change his or her lifestyle. Only about 10% of patients with DLE will go on to develop SLE.

Prevention

DLE cannot be prevented. Recommendations to prevent flares of DLE in patients with the disease include avoiding exposure to sun and consistently using sunscreen.

Key Terms

Antibody
Specialized cells of the immune system that can recognize organisms invading the body (like bacteria, viruses, and fungi). The antibodies are then able to start a complex chain of events designed to kill these foreign invaders.
Autoimmune disorder
A disorder in which the body's antibodies mistake the body's own tissues for foreign invaders. The immune system then attacks and causes damage to these tissues.
Immune system
The system of specialized organs, lymph nodes, and blood cells throughout the body that work together to defend the body against foreign invaders (bacteria, viruses, fungi, etc.).

Further Reading

For Your Information

    Books

  • Aaseng, Nathan. Autoimmune Diseases. New York: F. Watts, 1995.
  • Hahn, Bevra Hannahs. "Systemic Lupus Erythematosus." In Harrison's Principles of Internal Medicine, edited by Anthony S. Fauci, et al. New York: McGraw-Hill, 1998.
  • Long, James W. The Essential Guide to Chronic Illness. New York: HarperPerennial, 1997.
  • Wallace, Daniel J. The Lupus Book. New York: Oxford University Press, 1995.

    Periodicals

  • Mann, Judy. "The Harsh Realities of Lupus." The Washington Post, 120 (281)(October 8, 1997): C12.
  • Umansky, Diane. "Living with Lupus." American Health for Women, 16 (5)(June 1997): 92+.

    Organizations

  • American College of Rheumatology. 60 Executive Park S., Suite 150, Atlanta, GA 30329. (404) 633-3777. http://www.rheumatology.org.
  • Lupus Foundation of America, Inc. 1300 Piccard Dr., Suite 200K, Rockville, MD 20850. (800) 558-0121. http://www.lupus.org/lupus.

Gale Encyclopedia of Medicine. Gale Research, 1999.

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