How lupus affects the skin
Approximately two-thirds of people with lupus will observe some type of effect on their skin. In fact, 40-70 percent of people with lupus will find that their disease is made worse by exposure to ultraviolet (UV) rays from sunlight or artificial light.
Some individuals have or will develop a type of skin disease, called cutaneous lupus erythematosus. Skin disease in lupus can cause rashes or sores (lesions), most of which will appear on sun-exposed areas such as the face, ears, neck, arms, and legs.
A dermatologist, a physician who specializes in caring for the skin, should treat lupus skin rashes and lesions. He or she will usually examine tissue under a microscope to determine whether a lesion or rash is due to cutaneous lupus: taking the tissue sample is called a biopsy.
The Forms of Cutaneous Lupus
Lupus skin disease can occur in one of three forms:
- Chronic cutaneous (discoid) lupus
- Subacute cutaneous lupus
- Acute cutaneous lupus.
CHRONIC CUTANEOUS LUPUS (DISCOID LUPUS)
Discoid lupus appears as disk-shaped, round lesions. The sores usually appear on the scalp and face but sometimes they will occur on other parts of the body as well.
Approximately 10 percent of people with discoid lupus later develop lupus in other organ systems, but these people probably already had systemic lupus 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 the scalp may cause the hair to fall out. If the lesions form scars when they heal, the hair loss may be permanent.
Cancer can develop in discoid lesions that have existed for a long time. It’s important to speak with your doctor about any changes in the appearance 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 spent under indoor 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 the 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
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 the 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 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 with lupus:
Calcinosis is caused by a buildup of calcium deposits under the 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 the 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 the fingernail folds or on the tips of the 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 the fingers or around the 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 the scalp give a characteristic ragged appearance termed "lupus hair."
Raynaud’s phenomenon is a condition in which the blood vessels in the hands and/or 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 the fingers or toes to turn red, white, or blue. Pain, numbness, or tingling may also occur. People with Raynaud’s phenomenon should try to avoid cold conditions, and, if necessary, should 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 the capillaries and small arteries. A bluish, lace-like mottling will appear beneath the skin, especially on the legs, giving a "fishnet" appearance. Like Raynaud’s phenomenon, these conditions tend to be worse in cold weather.
Mucosal ulcerations are sores in the 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 the skin, especially on the lower legs, that result from low numbers of platelet in the 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 medications used to treat lupus-related skin conditions depends on the form of cutaneous lupus. The most common treatments are topical 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 immunomodulators, can treat serious skin conditions without the side effects found in corticosteroids: 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.
- Avoidance/protection from sunlight and artificial ultraviolet light
- Seek shade
- Sunscreens -- physical and chemical
- Corticosteroid creams, ointments, gels, solutions, lotions, sprays, foams
- Calcineurin inhibitors
- tacrolimus ointment (Protopic®)
- pimecrolimus cream (Elidel®)
Systemic Treatments for Mild to Moderate Disease
- Corticosteroids -- short term
- hydroxychloroquine (Plaquenil®)
- chloroquine (Aralen®)
- quinacrine (available from compounding pharmacies only)
- synthetic forms of vitamin A—isotretinoin (Accutane®), acitretin (Soriatane®)
- diaminodiphenylsulfone (Dapsone®)
Systemic Treatments for Severe Disease
- Corticosteroids -- long term
- oral—auronofin (Ridura®)
- intramuscular—gold sodium thiomaleate (Myochrisine®)
- Thalidomide (Thalomid®)
- Azathioprine (Imuran®)
- Mycophenolate mofetil (CellCept®)
- efalizumab (Raptiva®)
It should be noted that most of the above treatments are not approved by the Food and Drug Administration for cutaneous lupus.
is a Professor of Dermatology at University Hospital in Salt Lake City, UT and has specialized expertise in the diagnosis and treatment of skin changes that occur in Autoimmune Rheumatic diseases like Lupus.