The Skin
Approximately two-thirds of people with lupus will develop some type of skin disease. Skin disease in lupus can cause rashes or sores (lesions), most of which will appear on sun-exposed areas, such as your face, ears, neck, arms, and legs. In addition, 40-70 percent of people with systemic lupus will find that their disease is made worse by exposure to ultraviolet (UV) rays from sunlight or artificial light.
Lupus skin disease, called cutaneous lupus erythematosus, can occur in one of three forms: chronic cutaneous (discoid) lupus erythematosus, subacute cutaneous lupus erythematosus, and acute cutaneous lupus erythematosus. Lupus skin rashes and lesions should be treated by a dermatologist, a physician who specializes in caring for the skin. To determine whether a lesion or rash is due to cutaneous lupus, your physician will usually want to look at the tissue under a microscope. Taking the tissue sample is called a biopsy.
The Forms of Cutaneous Lupus
Chronic cutaneous lupus (discoid lupus) appears as disk-shaped, round lesions. The sores usually appear on your scalp and face, but sometimes they will occur on other parts of your body as well. Approximately 10 percent of people with discoid lupus later develop systemic lupus, but these people probably had systemic lupus to begin with, with the skin rash as the first symptom.
Discoid lupus lesions are often red, scaly, and thick. Usually they do not hurt or itch. Over time these lesions can produce scarring and skin discoloration (darkly colored and/or lightly colored areas). Discoid lesions that occur on your scalp may cause your hair to fall out. If the lesions form scars when they heal, your hair loss may be permanent. Cancer can develop in discoid lesions that you have had for a long time, so tell your doctor about any changes in the appearance of one of these lesions.
Discoid lupus lesions can be very photosensitive, so preventive measures are important:
- avoid being out in the sunlight between the hours of 10 a.m. and 4 p.m.
- use plenty of sunscreen when you are outdoors
- wear sun-protective clothing and broad-brimmed hats
- limit the amount of time you spend indoors under fluorescent lights
Subacute cutaneous lesions may appear as areas of red scaly skin with distinct edges, or as red, ring-shaped lesions. The lesions occur most commonly on the sun-exposed areas of your arms, shoulders, neck, and body. The lesions usually do not itch or scar, but they can become discolored. Subacute cutaneous lesions are also photosensitive so preventive measures should be taken when spending time outdoors or under fluorescent lights.
Acute cutaneous lupus lesions occur when your systemic lupus is active. The most typical form of acute cutaneous lupus is a malar rash -- flattened areas of red skin on your face that resemble a sunburn. When the rash appears on both cheeks and across the bridge of the nose in the shape of a butterfly, it is known as the "butterfly rash." However, the rash can also appear on your arms, legs, and body. These lesions tend to be very photosensitive. They typically do not produce scarring, although changes in skin color may occur.
Other Skin Problems
There are several other conditions that can occur when you have lupus:
Calcinosis is caused by a buildup of calcium deposits under your skin. These deposits can be painful, and may leak a white liquid. Calcinosis can develop from a reaction to steroid injections or as a result of kidney failure.
Cutaneous vasculitis lesions occur when inflammation damages the blood vessels in your skin. The lesions typically appear as small, red-purple spots and bumps on the lower legs; occasionally, larger knots (nodules) and ulcers can develop. Vasculitis lesions can also appear in the form of raised sores or as small red or purple lines or spots in your fingernail folds or on the tips of your fingers. In some cases cutaneous vasculitis can result in significant damage to skin tissue. Areas of dead skin can appear as sores or small black spots at the ends of your fingers or around your fingernails and toes, causing gangrene (death of soft tissues due to loss of blood supply).
Hair loss can occur for other reasons besides scarring on the scalp. Severe systemic lupus may cause a temporary pattern of hair loss that is then replaced by new hair growth. A severe lupus flare can result in fragile hair that breaks easily. Such broken hairs at the edge of your scalp give a characteristic ragged appearance termed "lupus hair."
Raynaud’s phenomenon is a condition in which the blood vessels in your hands and feet go into spasm, causing restricted blood flow. Lupus-related Raynaud’s usually results from inflammation of nerves or blood vessels and most often happens in cold temperatures, causing the tips of your fingers or toes to turn red, white, or blue. Pain, numbness, or tingling may also occur. If you have Raynaud’s phenomenon try to avoid cold conditions, and, if necessary, wear gloves or mittens and thick socks when in an air-conditioned area.
Livedo reticularis and palmar erythema are caused by abnormal rates of blood flow through your capillaries and small arteries. You will have a bluish, lacelike mottling under your skin, especially on your legs, that gives a "fishnet" appearance. Like Raynaud’s phenomenon, these conditions tend to be worse in cold weather.
Mucosal ulcerations are sores in your mouth or nose or, less often, in lining of vaginal tissue. These ulcers can be caused by both cutaneous lupus and systemic lupus. It is important to differentiate lupus ulcers from herpes lesions or cold sores, which may be brought on by the use of immunosuppressive drugs. Lupus ulcers are usually painless and signs of inflammation will show up in the biopsy.
Petechiae (pah-TEE-kee-eye) are tiny red spots on your skin, especially on the lower legs, that result from low numbers of platelet in your blood, a condition called thrombocytopenia. Although thrombocytopenia is common in lupus, serious bleeding as a result of the low number of platelets usually does not occur.
Treating Cutaneous Lupus
The medication used to treat lupus-related skin conditions depends on what kind of cutaneous lupus you have. The most common treatments are ointments, such as steroid cream or gel. In some cases liquid steroids will be injected directly into the lesions.
A new class of drugs, called topical immuno-modulators, has been developed to treat serious skin conditions without the side effects found in corticosteroids: both tacrolimus ointment (Protopic®) and pimecrolimus cream (Elidel®) have been shown to suppress the activity of the immune system in the skin, including the butterfly rash, subacute cutaneous lupus, and possibly even discoid lupus lesions.
In addition, thalidomide (Thalomid®) has been increasingly accepted as a treatment for the types of lupus that affect the skin; it has been shown to greatly improve cutaneous lupus that has not responded to other treatments.
Treatments for Cutaneous Lupus
Preventative Treatments Local/Topical Treatments Systemic Treatments for Mild to Moderate Disease Systemic Treatments for Severe Disease It should be noted that most of the above treatments are not FDA-approved for cutaneous lupus. Related Information Skin Issues and Hair Loss with Lupus The Many Faces of Cutaneous Lupus
15 Questions with Dr. David Fiorentino
Spring 2008 issue of Lupus Now magazine
15 Questions with Dr. Victoria Werth
Frequently Asked Questions
I want to avoid the butterfly red marks across the face. Is there anything I can do with lotions or creams to help avoid them?
There are several helpful approaches for this. First, patients prone to getting a butterfly rash should practice sun avoidance and use of high SPF sunscreens with good UVA blocks, such as Mexoryl or Antihelios. There are some anti-inflammatory creams, such as mild topical steroids, tacrolimus, or pimecrolimus than also can be of benefit in treating the red marks.
How might chemical peels affect someone with cutaneous lupus, and are they contraindicated for people with lupus?
Chemical peels may irritate the skin, and thus could potentially induce skin lesions in patients with the underlying disease. This phenomenon can occur with scratching or any skin irritation and thus could be a potential risk. This procedure should be discussed with your dermatologist before using it.
Is the use of tanning beds okay for people with lupus?
In general those with lupus should not use tanning beds. The bulbs in tanning beds produce ultraviolet light rays. It is the ultraviolet light rays that cause the skin to tan. The majority of people with lupus tend to be unusually sensitive to ultraviolet light. That is to say, exposure to excessive ultraviolet light, especially the UVB sun burning rays, can cause lupus skin lesions to appear, or make existing lupus skin lesions worse. Ultraviolet light can also activate the internal, or systemic, manifestations of lupus in some people.
There have been many examples of lupus skin disease patients who were thought to have psoriasis instead, and were then treated for psoriasis in medical phototherapy cabinets that are similar to tanning beds. A number of these people almost died from severe activation of their systemic lupus following such mistaken treatment.
Some research suggests that very long ultraviolet light wavelengths, in what is called the "UVA-1" range, can improve certain forms of lupus skin disease and mild forms of systemic lupus. This research has been somewhat controversial, because other research has found that higher doses of the same UVA-1 wavelengths are capable of aggravating the systemic manifestations of lupus. Therefore, the biological effects that people with lupus might experience (i.e. getting worse or better) are likely to be critically dependent upon the amount of the different wavelengths of ultraviolet light energy that their skin receives.
In a routine commercial tanning bed setting, it would be quite difficult for people with lupus to know how much of the various UV wavelengths they are receiving. Thus, for people with lupus, visiting a tanning salon may be too risky.
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