Patients with lupus erythematosus (LE) often have skin signs and develop many different types of lesions. LE skin diseases are divided into two broad categories: those skin lesions that occur only in people with LE, known as LE-specific skin lesions, and those skin lesions that can occur in people with LE, but can also occur in other diseases (LE-non-specific skin lesions). There are three broad categories of LE-specific skin lesions: chronic cutaneous LE (CCLE), or diseases limited to the skin, subacute cutaneous LE (SCLE), and acute cutaneous LE (ACLE). The most common lesion in CCLE is known as discoid LE; however, discoid skin lesions may appear in a patient with any type of LE.
What is the relationship between systemic LE (SLE) and chronic cutaneous (CCLE)?
LE can be viewed as a spectrum of disease, with DLE on one end, and full-blown systemic lupus erythematosus on the other end. Patients with DLE that is localized to the head and/or neck generally will not develop systemic disease, those with SCLE often have associated joint disease, and those with ACLE have active systemic disease. An individual patient may have limited skin disease and still have significant internal involvement.
What happens in LE?
Unlike people with SLE, people with discoid lesions usually do not produce autoantibodies, a reaction against substances in the body. 20-30% of the patients with SLE will develop specific skin lesions of lupus at some time, and 60-65% of people with SLE will develop some other type of skin problem. More severe forms involve the kidneys, brain, or other organs, but this is rare. A complete medical history, physical examination, and battery of tests can help in choosing the proper treatment.
Chronic Cutaneous LE (CCLE)
Discoid lupus (DLE) is the most common form of CCLE. The coin-shaped or disk-like “discoid,” lesions are mostly present on the scalp and face, but can be seen on other parts of the body. Patients with discoid lesions limited to the head, ears, and neck are classified as “localized DLE.” Patients with DLE discoid lesions on other body locations are classified as “generalized DLE;” some of these patients may rarely progress to SLE. DLE lesions are red, scaly, and thickened. With time, there can be scarring, atrophy (thinning), and discoloration of skin (darkly colored and/or lightly colored areas). When discoid lesions occur on the scalp, permanent hair loss (alopecia) can result. DLE lesions are usually painless and do not itch. Sun exposure may make lesions in CCLE patients worse. Skin cancer can occasionally develop in long-standing DLE lesions. Any changes should be brought to your dermatologist’s attention.
Other Forms of CCLE
When OLE lesions develop very thick scales, they are referred to as “hypertrophic” (thickened) or “verrucous” (wart-like) discoid lesions. Discoid lesions may also occur with firm lumps in the fatty tissue underlying the skin. This is called “lupus profundus” or “lupus erythematosus panniculitis” (LEP). LEP may be the only lesions that develop. OLE occasionally occurs in the inside surfaces of the mouth, nose, and eyes (mucosal OLE), and on the hands and feet (palmar-plantar OLE).
Subacute Cutaneous LE (SCLE)
There are two clinical forms of SCLE lesions, One type of SCLE looks like psoriasis and has red scaly patches on the arms, shoulders, neck, and trunk, with fewer patches on the face. The other type has red ring-shaped areas with a slight scale on the edges, Natural sunlight, as well as tanning beds, worsens both forms. The lesions of SCLE are not particularly itchy, and may heal with light or dark marks. Discoid lesions and lesions associated with ACLE can appear in SCLE patients.
Pregnancy and Lupus
The “neonatal LE syndrome” results when infants are born to mothers who have autoantibodies in their blood during pregnancy. Neonatal LE skin lesions usually disappear by six months of age. A small percentage of women with SCLE skin lesions are at risk for having a baby with neonatal LE or heart damage at birth (congenital heart block).
Acute Cutaneous LE (ACLE)
The most typical form of ACLE has flattened areas of red skin on the face that look like sunburn. When the cheeks and nose are involved, it is called a “butterfly rash.” Generalized ACLE can be seen on the arms, legs, and body. ACLE lesions are sunlight sensitive (photosensitive) and may discolor the skin, but there is no scarring. ACLE usually occurs in patients with active systemic disease.
Other Skin Lesions in LE (Non-Specific Skin Manifestations)
Vasculitis occurs because of damage to the blood vessels in the skin. This can appear as small red-purple spots or bumps on the lower legs. Occasionally, larger knots and ulcers can develop. Vasculitis may be hive-like or have small red or purple lines in the fingernail folds or on the tips of the fingers. This usually occurs in patients with active SLE.
Hair Loss (Alopecia) SLE patients who have been severely ill may develop temporary hair loss which is replaced by new hair growth. A severe flare of SLE can result in fragile hair that breaks easily. Broken hair at the edge of the scalp that has a ragged look is called “lupus hair.” When OLE involves the scalp, it should be treated promptly because damage to the hair follicle can cause irreversible hair loss.
Treatment Sunscreens are extremely important for people with LE. Prolonged periods of exposure to sunlight, especially between the hours of 10 am and 4 pm, should be avoided, as well as tanning parlors and even bare fluorescent light bulbs. In addition, wide-brimmed hats, tightly woven clothing, or sun protective clothing should be worn. A broad-spectrum sunscreen or block (SPF30) with protection against UVA and DYB rays should be reapplied every 2 hours.
ACLE is treated with systemic drugs such as prednisone, or in combination with other drugs that suppress the immune system. Discoid lesions and SCLE skin lesions can be treated with the application of corticosteroid creams, ointments, gels, tapes, and solutions. Individual lesions can be injected with a corticosteroid suspension.
Patients with more widespread LE skin lesions and stubborn lesions are treated with oral antimalarial drugs like hydroxychloroquine or in combination with quinacrine. Chloroquine may be used as a substitute for hydroxychloroquine.
Other oral drugs that are used include retinoids, prednisone, diaminodiphenylsulfone, gold, thalidomide, clofazirnine, or immunosuppressive drugs like methotrexate, azathioprine, mycophenolate mofetil, or cyclosporine.
Your dermatologist will provide necessary information in choosing the right treatment, as well as proper selection and use of sunscreens.