Facial discoid lupus
DLE is one of a large number of conditions occurring in a rare disease called systemic lupus erythematosus (SLE). Only a few people with DLE also have SLE. Other skin changes in SLE are photosensitivity (a rash on all sun exposed skin), mouth ulcers, and a curious ring-shaped rash. SLE may affect joints, kidneys, lungs, heart, liver and the blood. When tests are performed in a patient with DLE, usually no abnormalities are found. Sometimes however mild anemia or reduction in the number of circulating white cells is detected, and there may be some abnormal antibodies to cell nuclei (called antinuclear antibodies). The tests may need to be repeated every year or so. Rarely, people with DLE also have circulatory problems. They may have Raynaud's - this refers to abnormal blanching of fingers and toes in response to cold weather, followed by numbness and slow rewarming by the fingers which go blue then red. They may have mild arthritis in their hands.

Treatment
Reduce exposure to sunlight. Stay indoors whenever possible between 10 a.m. and 2 p.m. Cover up - wear a broad brimmed hat, long sleeves, high collar, long trousers or skirt, socks and shoes. Apply a Very High Protection Broad Spectrum sunscreen to all exposed skin. Ask your dermatologist which is most suitable for you. Topical steroids: Strong steroid creams should be applied accurately to the patches once or twice daily, until the patches have cleared up. Ask Dr. Jacobs how long it is safe to use your cream. A milder topical steroid can be used when the rash is less severe. Antimalarial tablets: DLE is not due to malaria. However, antimalarials (chloroquine, hydroxychloroquine) have anti-inflammatory properties that work well in most cases. Regular blood tests and eye checks are necessary. Oral steroids: Systemic steroid medicines are usually not necessary except in severe DLE.

What is the difference between discoid lupus and systemic lupus?
The term discoid is a very confusing term which, unfortunately, is inappropriately used by many people, including physicians. The term discoid simply means coin-shaped. The scarring coin-shaped lupus lesion commonly seen on areas of the skin that are exposed to light has been termed discoid lupus erythematosus. This term refers only to the description of the lupus lesion on the skin and should not be used to distinguish cutaneous lupus from systemic lupus erythematosus. A physician cannot determine whether or not a discoid lupus lesion on the skin is occurring in the presence or absence of systemic features just by examining the shape of the lesion. This can only be done by taking a complete history and physical examination and interpreting the results of appropriate blood tests.

What is the relationship between discoid and systemic lupus erythematosus? This is a common question. Lupus erythematosus should be viewed as a continuum of a spectrum of the disease. At one end of the spectrum, in its most mild form, it is characterized by coin-shaped, scarring skin lesions which we term discoid lesions. At the other end of the spectrum are those systemic lupus erythematosus patients who have no skin lesions, but have systemic features (i.e., arthritis or renal disease). People with only discoid lesions and no systemic features commonly have no auto-antibodies in their serum (i.e. antinuclear tests will be negative). On the other hand, people with systemic lupus erythematosus are characterized by the presence of one or more types of auto-antibodies in their blood. From personal experience and from reviewing the literature, it has been estimated that between 5 and 10% of patients initially presenting with only the coin-shaped lesions of discoid lupus will, with time, develop systemic features. As noted above, approximately 20% of people with systemic lupus erythematosus will at the time of the initial presentation of their disease have discoid lupus lesions. These data indicate that, at times, the lupus disease process is dynamic and, with time, a small percentage of those patients who only have discoid lupus lesions will eventually develop systemic disease. In addition to these coin-shaped, scarring lesions, there are several different types of discoid lupus lesions with which patients should be familiar. Occasionally, the discoid lupus lesions may occur in the scalp producing a scarring, localized baldness termed alopecia. At times, these discoid lesions may appear over the central portion of the face and nose producing a characteristic butterfly rash. This type of lupus obviously has significant cosmetic implications. The discoid lupus lesions may develop thick, scaly (hyperkeratotic) formations and are termed hyperkeratotic or hypertrophic cutaneous lupus lesions. Discoid lupus lesions may also occur in the presence of thickening (deep induration) of the layers of underlying skin. This is termed lupus profundus.

At the present time, research indicates that discoid lupus lesions are the result of an inflammatory process in the skin in which the patient's lymphocytes (predominantly T cells) play a major role. This is in contrast to systemic lupus erythematosus, where autoantibodies and immune complex formation are responsible for many of the symptoms.