Discoid Lupus Erythematosus (DLE) is the most benign form of lupus, in the majority of cases only affecting the skin.
The relationship between skin lesions and the development of systemic disease has been an area that has intrigued Rheumatologists and Dermatologists for many years. Some lupus patients have only skin problems, others have a combination of problems including renal disease and arthritis.
In general DLE is a benign disease, which rarely affects the internal organs, i.e. rarely becomes systemic. Most studies suggest that approximately 5% of patients with discoid lupus at some stage may suffer a generalised flare of the disease, involving joints, kidneys etc. and may progress to developing Systemic Lupus Erythematosus.What is discoid lupus?
Discoid lupus occurs due to inflammation of the skin, the cause of which is unknown. "Discoid" means coin-shaped. Discoid lupus can occur in patches across the body. These patches tend to be well defined, thickened and scaly, they are slightly red in colour and can itch. Characteristically, the skin most commonly affected is on the cheeks; there may be a "butterfly" distribution across the nose as in SLE, and the other skin areas commonly affected are the palms of the hands and soles of the feet (reddish, sometimes peeling lesions), the elbows, ears and the scalp. Each patch is called a "plaque" and tends to occur on sun or light exposed areas such as face, neck, ears and forearms. If discoid lupus occurs on the scalp it often results in the destruction of the hair follicle with consequent hair loss. Occasionally, if the skin is inflamed the patient may suffer with conjunctivitis and mouth ulcers. The appearance of patches can vary on different areas of the body and between patients. As these patches heal they may leave scarring and, on darker skins, the pigment in the skin can be lost leaving white areas.
Discoid lupus can appear similar to other skin lesions such as rosacea (which appears basically as red cheeks and a red nose), fungal infections, sarcoidosis, seborrhea, dermatoymyositis and a sun-sensitive rash called polymorphous light eruption. These can be ruled out by a simple skin biopsy and blood tests before diagnosing DLE. There are other skin lesions that can be seen in discoid lupus, although many are rare. These include verrucous (warty) or hypertrophic (thickened) lesions, lace-like patterns and chilblain-like discoid lupus. There is a fairly rare type called Lupus Erythematosus Profundus, which is an inflammation of the fat under the skin.
Although the disease may not be life-threatening there are a number of problems:
Lupus Erythematosus should be viewed as a spectrum of disease. At the mild end of the spectrum it is characterized by coin-shaped, possibly scarring, skin lesions which we term discoid lesions. At the other end of the spectrum are those Systemic Lupus Erythematosus patients whose predominant features are those of systemic disease (e.g. arthritis or renal disease). Patients with only discoid lesions and no systemic features commonly have no auto-antibodies in their serum (i.e. antinuclear or anti-DNA tests will be negative) whilst patients with Systemic Lupus Erythematosus are characterized by the presence of one or more types of auto-antibodies in their blood.
If the discoid lesions appear only above the neck, they rarely evolve into systemic lupus and are treated with antimalarial drugs or local remedies. This is sometimes called localised DLE. In other cases many lesions are present on the arms, legs and trunk as well as the head and neck. This is called generalised DLE and has a 10% chance of developing into systemic lupus.Treatment
The disease needs treating as the scarring produced by it can be cosmetically disfiguring. There is a minor risk of the development of skin cancer within the old scars. However, this is rare and with ongoing treatment and consultant supervision there is no cause for alarm.
The British Red Cross and some hospital Dermatology clinics have a free Cosmetic Camouflage service which can be extremely helpful to patients conscious of scarring or redness.
In both systemic and discoid lupus, signs and symptoms may often be due to photosensitivity - a reaction to the sun. Sunlight is well known to exacerbate a flare. For patients with systemic lupus, protection from sunlight is recommended, but for those with discoid lupus it is vital. Avoiding sunlight is important because ultraviolet light changes the DNA cells and, therefore, accelerates the skin lesions. Sunscreens should be of a high sun-protective factor (HPF 15+) - anything below this will not fully protect the skin. Sunscreens will not last the whole day and must be reapplied frequently. If the skin is very sensitive, the purchase of treated film to fix to windows in the home and in the car should be considered. [See also the factsheet on "Lupus and Light Sensitivity".]Drugs
Corticosteroids used topically (on the skin) are often quite effective, especially for discoid lupus. More chronic lesions are often treated with non-fluorinated steroids such as hydrocortisone ointment, which is quite weak. In order to flatten and weaken a thick plaque a stronger treatment may be needed such as a fluorinated steroid, Diprosone or Betnovate, returning to the weaker ointment when the plaque flattens.
Ointments are very effective, but many patients find them less acceptable during the day as they tend to be greasy. Cortisone can be made up into an injectable substance and put into a lesion, especially those which are warty or very thick. Occasionally, the use of oral steroids may be necessary. These are used for other problems in lupus if the internal organs are affected but are rarely used if there are skin signs alone.
Antimalarial treatment can also be used. Hydroxychloroquine has been found to be very effective in a high percentage of discoid patients. Patients given antimalarials will have a baseline assessment by the prescribing physician, and thereafter annual evaluations. Referral to an ophthalmologist will be made if the patient has visual impairment or eye disease at baseline assessment or if there is a change in vision whilst on treatment. Years ago, there was a problem of the drug being deposited in the retina and serious eye problems developing; however, the low doses being used now have virtually eliminated this risk. Patients are advised, though, to err on the side of caution and have an annual eye check-up.The physician’s goal in treating discoid lupus is to:
©Lupus UK. Last updated 6 August 2012.