Lupus Erythematosus

Lupus erythematosus is an autoimmune disease, that is, a disease that alters the function of your immune system. No one knows what causes lupus erythematosus, but it is defined by a list of clinical characteristics associated with the production of autoantibodies, which are antibodies that are produced by your immune system. This is a disease with symptoms that vary widely in their severity from limited skin disease at one end of the spectrum to life threatening disease that invades other organs in your body at the other. We will focus here on the skin problems you could experience with lupus erythematosus. There are different types of skin involvement in Lupus Erythematosus.

Lupus Erythematosus – Specific Skin Disease:

These are scarring, coin-shaped lesions commonly seen in areas of skin that are exposed to light, such as the scalp and ears, and the central portion of the face and nose. More rarely, your lips, mouth, and tongue might be involved. These lesions can produce a scarring baldness, and because they often affect your face, you may consider getting cosmetic treatment. Only 1 in 10 to 1 in 20 of patients who are initially diagnosed with this type of skin involvement will eventually develop the severe form of the disease that involves other organs in your body.

These are non-scarring, red and scaly lesions that are very photosensitive, that is they get worse when they are exposed to ultraviolet light. They tend to occur on the face in a butterfly shaped distribution or can be more widespread on the body. Even though these lesions do not result in scarring, their extent and color change can cause you major cosmetic concerns. About half of the patients who are diagnosed with this type of skin involvement will, in time, develop other organ involvement (also called systemic involvement or systemic disease), such as arthritis conditions with their blood. Kidney disease is unusual in patients with this type of skin disease.

This is a rare type of skin lupus erythematosus in which the subcutaneous fat is involved giving rise at first to tender nodules that can leave, in time, saucer like depressions in the skin surface. This type most commonly affects the upper arms and trunk. Patients with this type of skin involvement may have either systemic disease or disease limited to the skin.

The malar rash or butterfly rash occurs in association with systemic lupus erythematosus. It is seen in up to 2/3 of patients with systemic disease and may be the presenting feature in up to 40%. It may vary in degree from a mild redness or “rosy cheeks”, to multiple swollen red areas or plaques.

Lupus Erythematosus – Non-Specific Skin Disease:

The following are skin changes that are noted in some patients with lupus erytheamtosus but also occur in many patients without lupus erythematosus. Thus having one of the conditions listed below does not mean that there is a predisposition to developing lupus erythematosus.

Up to 1/3 of patients with systemic lupus erythematosus get reversible form of hair loss associated with flares of their systemic disease. They may also note, that their hair is more brittle than previously and breaks easily giving rise to shortened hair (“lupus hair”). Another form of reversible hair loss that leaves distinct bald spots, alopecia areata, may also be more common in patients with lupus erythematosus.

Patients with systemic lupus erythematosus may develop inflammation of their blood vessels. This can result in varied manifestations running a spectrum from multiple scattered red bumps, that may crust and ulcerate to painful nodules.

This is the blanching of the skin of the fingers and toes when exposed to cold and may be followed by a blue or red discoloration.

This is a cold induced injury that results in tender nodules and plaques on the fingers and toes.

Frequently Asked Questions:

The specific skin forms of lupus erythematosus have a characteristic appearance. To confirm the diagnosis, your doctor may perform a skin biopsy of affected skin. Examination of a small sample of your skin under the microscope will allow a more definite diagnosis as the microscopic tissue changes are characteristic. In addition, a small sample may be obtained for an immunofluorescence test. Lupus erythematosus is a condition in which there is antibody production to self-tissues, and these may be detected in the skin with this test.

Discoid lupus erythematosus can mimic many other skin diseases including psoriasis, fungal infection of the skin, and other rare inflammatory skin disorders. Subacute cutaneous lupus erythematosus can also mimic psoriasis and a common form of light sensitivity called polymorphous light eruption. In this condition, itchy bumps or welts may appear within minutes to hours after sun exposure. The malar rash of acute cutaneous lupus erythematosus can mimic rosacea, a common condition also causing redness of the cheeks. As skin lupus erythematosus can mimic many common skin conditions, a skin sample or biopsy is often required to confirm the diagnosis.

The subacute cutaneous form of lupus erythematosus can be caused by certain medications. In addition, patients with cutaneous lupus erythematosus can have their skin disease worsened by certain medications. These are rare, unpredictable side effects of these medications and in almost all such cases the association with the skin disease is only suspected and not proven. The list of suspected drugs includes certain diuretics (hydrochlorothiazide), anti-inflammatory drugs (piroxicam, naproxen, oxyprenolol), calcium antagonists used in the control of blood pressure (diltiazem), and anti-fungal agents (terbinafine). If the suspected drug is a culprit, discontinuation of the drug should result in improvement of the skin disease.

The risk of developing systemic lupus erythematosus depends of the specific form of cutaneous lupus erythematosus that is diagnosed. Only one in 10 to 20 of patients presenting with classic discoid lupus erythematosus will go on to develop systemic disease. Approximately 50% of patients presenting with subacute cutaneous lupus erythematosus or lupus profundus will develop relatively mild forms of systemic lupus erythematosus.

The skin manifestations of lupus erythematosus are the result of inflammation in the skin that is primarily mediated by inflammatory cells called T lymphocytes. How and why these T cells are activated to cause disease is still unclear. Contributing factors include a genetic predisposition and environmental factors. Genetic factors - These genes encode proteins that are important in controlling the immune system and in fighting infection. Ultraviolet light is an environmental factor that can have an adverse effect both on skin lupus and systemic lupus erythematosus. It is thought that ultraviolet light can increase cell death in the skin and thereby boost the immune response to self. Ultraviolet light can also alter the responses of the immune system itself to antigens.

What Can You Do About Your Lupus Erythematosus?

The goals of treatment are to control and stop the development of new lesions, so as to minimize and prevent scarring and disfigurement. In addition, scars can be appropriately managed to minimize their cosmetic impact.

Sunlight:

The specific lesions of cutaneous lupus erythematosus occur on sun-exposed skin. In addition a significant proportion ( up to 70%) of patients report that their skin disease and possibly even systemic disease is aggravated by sunlight exposure. Ultraviolet light in both the sunburn (UVB) and long wave spectrum (so called black light or UVA) can cause lupus lesions to appear. It is important to note that long wave ultraviolet light is not blocked by window glass, and a number of patients are so photosensitive that they will burn through window glass.

Consequently, sun protection can do a lot to minimize the development of new lesions. Exposure to sunlight can be minimized by avoiding sunlight from 10 in the morning to 3 in the afternoon. Further protective clothing, that blocks sunlight and the wearing of wide-brimmed hats can minimize exposure. The regular use of a sunscreen that blocks both UVA and UVB is recommended. These sunscreens should be applied even on cloudy days as UVA can easily penetrate clouds. An SPF (sun protection factor) of at least 15 and as high as 60 is recommended. A number of sunscreen preparations have been specifically tested for the prevention of the induction of skin lupus erythematosus lesions and these can be obtained from your dermatologist.

Smoking:

Recent studies have shown that smoking increases the risk of developing cutaneous lesions of lupus erythematosus. Further, smoking decreases the effectiveness of standard drug treatment of cutaneous lupus erythematosus.

Trauma:

The specific skin lesions of lupus erythematosus can spread to areas of freshly damaged skin. In this way, lesions may spread to areas of previous sunburn or to areas that have undergone recent surgery. Likewise the picking or traumatizing of lesions can cause their spread.

Cosmetics can be used to minimize the impact of established lesions, camouflage cosmetics such as Covermark can efficiently minimize discoloration.

Topical and local treatment:

Systemic treatment:

In the presence of advancing disease despite local therapy or in the presence of widespread disease, systemic therapy is considered.

First line systemic therapy consists of the use of antimalarial drugs. If the skin disease is resistant to a single antimalarial drug, a combination of antimalarial drugs is often used next.

Second line therapy consists of the use of other drugs that have been shown to modulate the immune system such as the vitamin A derivatives (isotretinoin or acitretin) or dapsone.

If these agent fail, more potent immunosuppressive drugs are used on rare occasion. These may include methotrexate, azathioprine, mofetil mycophenolate, cyclosporine, systemic corticosteroids, and thalidomide.

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