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How Lupus Affects the Skin  

About a third of all people with lupus will develop some form of skin disease, according to the Lupus Foundation of America. Of these, 40 to 70 percent will find that exposure to ultraviolet (UV) rays makes their disease worse.

Some people with lupus develop cutaneous lupus erythematosus, a skin condition that causes rashes or sores on areas of skin exposed to the sun, such as their face, ears, neck, arms, and legs.

As physicians who specialize in caring for the skin, dermatologists can treat lupus rashes and sores, also known as skin lesions. Dermatologists sometimes take a small sample of skin, known as a biopsy, for examination under a microscope to determine if the rash or lesion was the result of lupus.

Types of Cutaneous Lupus Erythematosus

There are several types of cutaneous lupus erythematosus; each causes different symptoms and requires different treatment.

Chronic cutaneous (discoid) lupus

Discoid lupus appears as disk-shaped, round lesions that usually appear on the scalp and face, although they can develop on skin elsewhere on the body. These lesions are usually thick, red, and scaly. These skin lesions do not hurt or itch, but they can cause scarring and discolorations of the skin. When the lesions occur on the scalp, they can cause hair to fall out; hair loss may be permanent if the lesions form scars on the scalp.

About one in ten people with this type of lupus develop lupus in other organ systems. While rare, cancer can develop in long-lasting discoid lesions. If you notice any changes in the appearance of these lesions, speak with your doctor.

Discoid lupus lesions can be very photosensitive, which means exposure to sunlight can cause severe rashes. You can reduce your risk for flare-ups by taking preventive measures, which include:

  • Avoiding sunlight between 10 am and 4 pm
  • Using plenty of sunscreen with an SPF of 70 or higher when you are outdoors
  • Wearing broad-brimmed hats and sun-protective clothing
  • Limiting the time you spend under indoor fluorescent lights

Subacute cutaneous

Subacute cutaneous lesions have a scaly ring-like appearance. They develop most commonly on the areas of the body exposed to sunlight. These lesions do not itch or scar, but they can discolor the skin. Subacute cutaneous lesions are also photosensitive, so you should take the same precautions as discoid lupus lesions.

Acute cutaneous lupus

Acute cutaneous lupus lesions may develop when your systemic lupus is active. It often appears as a sunburn-like butterfly-shaped rash over the cheeks and bridge of the nose, but it can appear elsewhere on your body. While they do not usually produce scarring, changes in skin color may occur. They typically do not produce scarring, although changes in skin color may occur.

Other skin conditions
Calcinosis

Calcinosis is the buildup of calcium deposits beneath the skin. They can develop as the result of kidney failure; calcinosis may also develop as a reaction to steroid injections, which are often given as a treatment for lupus flares. Calcinosis can be painful and they may leak a white, chalky fluid if they break through the skin.

Cutaneous vasculitis lesions

Lupus causes inflammation, which can damage blood vessels in the skin. Cutaneous vasculitis lesions can develop as a result of this damage. The lesions usually appear as small, red/purple spots and bumps on the lower legs, but larger nodules and sores can develop. Cutaneous vasculitis lesions may also look like raised sores, or as red/purple lines or spots in the fingernails or fingertips. The condition may also cause significant damage to skin – patches of dead skin, appearing as small black spots or sores, may develop at the tips of the fingers and around fingernails and the toes. Loss of blood supply to the area can cause soft tissue death, also known as gangrene.

Hair loss

While scarring of the scalp is a common cause of hair loss in people with lupus, severe systemic lupus can cause temporary hair loss and severe lupus flares can cause fragile hair that breaks easily. Broken hairs at the ends of the scalp create a rugged appearance to the hairline.

Raynaud’s phenomenon

Raynaud’s phenomenon is a condition in which the blood vessels in the hands and/or feet spasm, which restricts the flow of blood to tissues there. Lupus can lead to Raynaud’s phenomenon through inflammation of the nerves or blood vessels, which causes the fingertips or toes to turn red, white, or blue; pain, tingling, or numbness may occur. Since cold temperatures can trigger Raynaud’s phenomenon, people with lupus who experience the phenomenon should avoid cold temperatures and consider wearing thick socks and gloves or mittens when in air-conditioned environments.

Livedo reticularis and palmar erythema

Palmar erythema and livedo reticularis are circulation problems that affect the skin and often accompany lupus. Caused by swollen blood vessels, livedo reticularis results in a reddish-blue fishnet-like pattern that typically develops on the legs. Palmar erythema occurs when tiny blood vessels, known as capillaries, widen to cause the bases of the palm, thumb, and little finger – and sometimes all of the fingers – to turn red. As with Raynaud’s phenomenon, cold weather can trigger livedo reticularis and palmar erythema.

Mucosal ulcerations

Sores, known as mucosal ulcerations, may develop in the mouth or nose; less often, they appear in the lining of vaginal tissue. Both cutaneous lupus and systemic lupus can trigger mucosal ulcerations. It is important to determine the cause of these sores, as they can look similar to herpes lesions and cold sores brought about by the use of immunosuppressive drugs.

Petechiae

Tiny red spots in the skin, known as petechiae, are the results of low platelet numbers. Platelets are the components of blood that help slow bleeding. Low platelet counts are common in lupus, but serious bleeding problems rarely occur.

Treating Lupus Skin Conditions

Dermatologists prescribe various medications to treat lupus-related skin conditions, depending on the type of cutaneous lupus. They commonly prescribe topical ointments, such as creams and gels, but sometimes suggest the injection of liquid steroids directly into the lesions.

Corticosteroids effectively treat many of the serious skin conditions associated with lupus, but steroids may cause unpleasant side effects. Topical immunomodulators are a new class of drugs that can treat serious lupus-related skin conditions, such as butterfly rash, subacute cutaneous lupus, and sometimes discoid lupus lesions, without the side effects of steroid treatment. These drugs, such as tacrolimus ointment (Protopic®) and pimecrolimus cream (Elidel®) have been shown to suppress the activity of the immune system and relieve these skin conditions. Use of the drug thalidomide (Thalomid®) is becoming more commonplace, as research shows it improves cutaneous lupus that has not responded to other treatments.

Treatment approaches

*The US Food and Drug Administration (FDA) may not have approved all of these treatments for cutaneous lupus.

Preventive measures
  • Avoid ultraviolet light from sunlight and artificial sources
  • Wear protective clothing
  • Seek shade
  • Apply sunscreen

Local/topical treatments
  • Corticosteroid creams, gels, ointments, solutions, sprays, lotions, foams
  • Calcineurin inhibitors such as:
    • Pimecrolimus cream (Elidel®)
    • Tacrolimus ointment (Protopic®)

Systemic treatments for mild to moderate disease
  • Corticosteroids (short course)
  • Antimalarials such as:
    • Chloroquine (Aralen®)
    • Hydroxychloroquine (Plaquenil®)
    • Quinacrine (available only through compounding pharmacies)
  • Diaminodiphenylsulfone (Dapsone®)
  • Retinoids
  • Sulfones
  • Synthetic forms of vitamin A—isotretinoin (Accutane®), acitretin (Soriatane®)

Systemic treatments for severe disease
  • Corticosteroids – long course
  • Gold 
    • Intramuscular— gold sodium thiomalate (Myochrysine®)
    • Oral—auranofin (Ridura®)
  • Azathioprine (Imuran®)
  • Biologics
  • Efalizumab (Raptiva®)
  • Methotrexate
  • Mycophenolate mofetil (CellCept®)
  • Thalidomide (Thalomid®)
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