Skin Changes in People with Systemic Lupus

Introduction

Changes of the skin, hair, nails, and mucous membranes are very common in people with systemic lupus erythematosus (SLE), occurring in 85 percent of patients. In most of them, these abnormalities are not severe and are easily treated with corticosteroids (prednisone) or antimalarial drugs (hydroxychloroquine or chloroquine). In a small number of individuals, these problems are severe and may be somewhat unresponsive to the usual treatment. The rashes of SLE may be divided into specific and non-specific rashes.

Specific rashes

1. The butterfly blush or rash is a faint or prominent red rash over the malar area and the bridge of the nose. The rash does not go into the sides of the nostrils or down the fold between the nose and the outer part of the lips. These areas are always spared and look white in contrast to the red rash of the cheeks and bridge of the nose. Sometimes the rash is flat, and sometimes it is elevated. It may be in the form of red blotches or may be completely red over the area. The rash may be itchy, especially if it looks more like a rash than a blush. This rash is typical of SLE but is present in only about 30 percent of patients.
   
   The butterfly rash is frequently confused by patients and by physicians with a similar red rash which also is over the cheeks. This rash is called acne rosacea. It does involve the areas of the outside of the nostrils and does involve the folds between the nose and the outer part of the lips. In addition, pimples may be seen on top of the red rash in acne rosacea.

     2) Subacute cutaneous lupus lesions are of two major types.

• The first type looks like red pimples when the rash first comes out; as the rash persists, these pimples become bigger, and scales appear as the rash persists. Patients complain about the rash being very itchy. The rash usually appears on the face, chest, or arms and commonly comes on after sun exposure. The rash usually worsens after more sun exposure.
  
  
• The second type starts out as a flat lesion and gets bigger by expanding outward. The center may become less red and may even clear up completely so that, after a while, this rash looks like many circular red areas with clear holes in their centers. The rash appears on the face, chest, arms, and back. It is very sensitive to the sun and, like the other form of subacute cutaneous lesions, usually is very itchy. The rash of subacute cutaneous lupus usually heals without scarring, or leaves a non-depressed scar or area of depigmentation where the rash had been.

People with subacute lesions are very sun-sensitive. These individuals also frequently have a specific nuclear autoantibody called anti-Ro. SLE patients with anti-Ro are more likely to have a sun-sensitive rash than SLE patients without anti-Ro.

3. Chronic discoid lupus lesions are found in about 20 percent of people with SLE. Chronic discoid lupus also is found in people who have absolutely no trace of systemic lupus. In these people, the lupus is confined to the skin.
   
   These lesions are slightly elevated, pink or red areas which form crust or flakes on the surface. As the lesions mature, the central area becomes depressed and forms a scar. These lesions rarely are found below the chin and practically never on the legs. However, chronic discoid lupus frequently is found on the scalp and in the outer ear. Lesions are itchy and get bigger by spreading outward, leaving a central scar. In pigmented individuals, the central area may become depigmented; in all individuals, the outer red area may become hyperpigmented. These can be very disfiguring lesions and should be treated as soon as possible and as aggressively as necessary to stop them.

Non-specific rashes

Non-specific rashes are seen in diseases other than lupus, but are extremely common in people with SLE. Patients may develop a red blotchy rash similar to the rash caused by a number of viruses, and similar to the rash caused by a variety of drugs (so-called drug-rash). These blotches may or may not be elevated and may be pink or red. They generally are found on the face, chest, back, and upper arms, and may or may not be itchy. This type of rash disappears quickly if patients are treated with corticosteroids for their other complaints.

Vasculitic rashes are seen frequently in people with SLE, especially in acutely ill patients. These rashes are non-specific because they may be present in patients who do not have lupus. For example, a small number of individuals with rheumatoid arthritis develop vasculitic rashes. There are a wide variety of rashes due to vasculitis, including: tender bumps in the tips of the fingers or toes; rashes that look like splinters under the nailbeds; ulcers that form around the ankle joints or on the legs; and tender red bumps on the shins.

Some lesions in people with lupus, such as hives, may be due to inflammation of the small vessels of the skin.

Other non-specific lesions include nail bed redness and redness of the palms of the hands. Rarely, blisters due to lupus may be present.

Hair loss (alopecia)

Hair loss occurs in about 45 percent of people with lupus at some time during the course of their disease. Most frequently the hair loss occurs at the onset of the illness, and may be one of the first symptoms of the disease recognized by the person. Most often, the hair loss is from all over the head, but sometimes the hair falls out in patches. When the disease is brought under control, the hair should grow back. Sometimes there is a rash in the scalp—usually subacute or chronic discoid—that interferes with the hair follicle. In this situation, the individual is left with a permanent area of alopecia. Drugs used to treat lupus, such as prednisone and immunosuppressive therapies, also may be the cause of reversible hair loss.

Nail changes

Nail changes occur in about 10 percent of patients and consist of cracking, curling, and even loss of nails. These symptoms are due to the changes in the small vessels of the nail bed.

Mucosal ulcers

Mucosal ulcers usually are found on the roof of the mouth. They are painless, but are found because the physician looks for them. Patients may mistake painful canker sores for mucosal ulcers. Some individuals with lupus will have mucosal ulcers in the nose - when these are severe, they can lead to a hole in the septum of the nose.

Treatment

The mainstay of treatment is the use of antimalarial drugs such as hydroxychloroquine (Plaquenil). Some people are more responsive to chloroquine. Steroid creams should be used cautiously on the face since the strong creams may cause dilated blood vessels. Some lesions—particularly discoid lesions—can be injected with corticosteroids and may respond very well. Other agents that have been used include retinoids such as Accutane and thalidomide. Occasionally it is necessary to use moderate doses of corticosteroids or other immunosuppressive therapies orally, to control lesions. Vasculitic lesions may require the use of cyclophosphamide, especially when ulceration is present.

There also are a number of practical, general steps people with lupus can take to help their skin problems.

1. The first rule is to stay out of the sun, especially during the middle of the day.
2. The second rule is to wear a good protective sunscreen of SPF 30 or higher. Use the sunscreen on all exposed skin areas, including the hands.
3. The third rule is to wear a hat with a broad brim.
4. The fourth rule is to wear long sleeves.

 

 

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