Lupus and Skin Diseases

kin disease is a very common symptom of systemic lupus erythematosus (SLE), or lupus. Rashes are often the first visible indication of the disease. Eighty per cent of lupus patients will develop some kind of skin manifestation throughout the course of their disease.

There are numerous rashes associated with lupus, but there are three main types:

1. Acute Cutaneous Lupus Erythematosus (ACLE)
2. Subacute Cutaneous Lupus Erythematosus (SCLE)
3. Discoid Lupus Erythematosus (DLE), sometimes referred to as Chronic Cutaneous Lupus Erythematosus (CCLE).

Acute Cutaneous Lupus Erythematosus (ACLE)

Also known as:

• “Malar rash,” referring to the involvement of the upper cheeks.
• “Butterfly rash,” referring to the unique butterfly-like shape. ACLE is the most common skin manifestation of lupus and is often referred to as the “lupus mask” due to its distinctive shape and the frequency with which it is seen in patients.

Characteristics of ACLE:

• Symmetrical (uniform) appearance that covers both cheeks and the bridge of the nose.
• In some cases, the neck and forehead are also affected.
• Skin is red and swollen, similar to a sunburn.
• The rash is persistent, as opposed to other skin changes that last only minutes or hours.

Causes and Long-term Effects:

The precise cause of ACLE is unknown. However, underlying lupus activity can trigger the rash. As is explained below, ACLE is photosensitive, meaning that exposure to ultraviolet (UV) light, including the sun, can trigger an outbreak. (Sun exposure can also trigger or worsen a general lupus flare.) An ACLE outbreak can last for weeks or longer but once the rash clears, there are usually no permanent effects.

Subacute Cutaneous Lupus Erythematosus (SCLE)

Characteristics of SCLE

• Ring-shaped or disk-shaped patches of red, crusty skin.
• Most often develops on skin that has been exposed to the sun or another form of ultraviolet light.

Causes and Long-term Effects

As with most skin diseases associated with lupus, SCLE is photosensitive – it is triggered or worsened by ultraviolet (UV) light. Some patients experience SCLE lesions as a side effect of medications. This type of SCLE usually clears up once the patient stops taking the medication and traditional SCLE treatments with antimalarial medications and corticosteroids begin. Lesions usually heal without scarring, but some skin lightening or darkening can occur.

Discoid Lupus Erythematosus (DLE)

DLE is also known as Chronic Cutaneous Lupus Erythematosus (CCLE). Patients with DLE only develop skin disease and do not exhibit other symptoms associated with the disease, such as arthritis.

There are two types of DLE: localized DLE, in which the lesions are primarily located on the scalp and face, and generalized DLE, in which lesions can be located anywhere on the body.

Characteristics of DLE

• Only affects the skin.
• Disk or coin-shaped scaly lesions (similar to SCLE).
• Generally found on the scalp and face, particularly the cheeks and nose.
• Usually painless.

Causes and Long-term Effects

Research suggests that patients with DLE are more photosensitive than SLE patients. Because DLE lesions can lead to scarring, it is important for patients to minimize sun exposure whenever possible. If this scarring occurs on the scalp, it can damage hair follicles and lead to permanent hair loss. A small percentage of people with DLE (five to ten per cent), will go on to develop SLE. It is important to monitor DLE symptoms and discuss any changes with your health care provider. Every individual with lupus is different and the stimuli that cause a flare will differ from patient to patient. While some causes are common, (sunlight for example), others can be as varied as the individual. Medications, environmental factors and any other number of things can stimulate symptoms. By paying attention to their symptoms, lupus patients may be able to identify triggers and learn to avoid them.

Treatments

Topical treatments, such as corticosteroid creams, applied directly to the affected skin are often effective, and may be the only treatment needed if rash is the only symptom. To treat persistent or aggressive rashes, corticosteroids can be injected directly into the skin. Anti-malarial medications (chloroquine [Aralen™], hydroxychloroquine [Plaquenil™]) are also highly effective in treating skin disease (see Fact Sheet on medications in lupus). Patients with systemic lupus and/or lupus of the skin should avoid direct sunlight and ultraviolet (UV) light whenever possible, and when going outdoors, they should take precautions against sun exposure, such as using sunscreen (sun protective factor [SPF] 30 or greater) and covering their skin.