Skin disease-also called cutaneous disease-is very common in lupus erythematosus (LE). Only joint pain (arthritis) is more common. People with lupus can develop many different types of skin lesions. The term "skin lesion" used here refers to a distinct area of abnormal skin.
Lupus skin disease can be divided into two broad categories:
1. Skin lesions that are seen only in people with LE. These three types of lesions are:
* chronic cutaneous LE (CCLE), also called discoid LE (DLE)
* subacute cutaneous LE (SCLE)
* acute cutaneous LE (ACLE).
2. Skin lesions that can occur in people with LE but can also occur in other diseases. There are a number of forms of skin disease in this category, such as:
* calcinosis (calcium deposits in the skin)
* hair loss (alopecia)
* rheumatoid nodules
* Raynaud's phenomenon
* livedo reticularis.
Systemic Lupus And Discoid Lupus
A frequently asked question by people with lupus is: "What is the relationship between systemic LE (SLE) and lesions seen in discoid LE (DLE)?"
Lupus can be viewed as a disease continuum or spectrum:
* At the mild end of the spectrum are the minimally affected LE patients. They will have only localized DLE skin lesions.
* At the other end of the spectrum are patients who have active SLE with internal organ involvement. They will have ACLE skin lesions or no skin lesions at all.
* SCLE patients tend to fall toward the middle of the spectrum since they often have some evidence of mild SLE.
People with DLE lesions and no clinical evidence of SLE usually do not produce autoantibodies in their blood (i.e. antinuclear and anti-DNA autoantibody tests are negative). On the other hand, SLE is characterized by the presence of one or more types of such autoantibodies.
* Of the patients who initially have only DLE lesions, approximately 10 percent will go on to develop SLE.
* Of the patients with SLE, approximately 20-30 percent will develop DLE lesions at some time during the course of their disease.
Whether DLE lesions are occurring alone or as a result of SLE can only be determined by:
1. performing a complete medical history
2. physical examination
3. a set of appropriate blood tests.
* Approximately 20 percent of people with SLE will have DLE as their initial disease symptom.
* An additional 10 percent of people with SLE will have subacute cutaneous LE (SCLE) skin lesions as their initial disease symptom.
* In addition, approximately 60-65 percent of people with SLE will develop some type of skin problem during the course of their illness.
However, skin lesions in people with lupus are less of a problem with the use of:
* oral corticosteroids (steroids) such as prednisone
* antimalarial drugs such as hydroxychloroquine (brand names: Plaquenil, Quineprox).
Forms Of Chronic Cutaneous Lupus Erythematosus (CCLE)
DLE is the most common form of CCLE.
* The coin-shaped ( i.e., "discoid") lesions of DLE are most commonly seen on the scalp and face, but can be seen on other parts of the body as well.
o Localized DLE: lesions limited to the head, ears, and neck
o Generalized DLE: lesions present both above and below the neck (generalized DLE carries a slightly increased risk of accompanying SLE).
* DLE lesions are often red, scaly, and thickened. As the lesions get older they can produce scarring and discoloration of skin (darkly colored and/or lightly colored areas).
* When DLE lesions occur in the scalp, hair loss (alopecia) can result. This form of hair loss is often permanent.
* DLE lesions are usually painless and typically do not itch.
* Skin cancer can occasionally develop in long-standing DLE lesions; therefore, any changes in a long-standing DLE lesion should be brought to your doctor's attention.
Other forms of CCLE
Hypertrophic or verrucous
* DLE lesions that develop very thick scale (hyperkeratosis) are referred to as:
o hypertrophic (thickened) lesions OR
o verrucous (wart-like) lesions.
* DLE lesions may also occur in conjunction with firm lumps in the fatty tissue underlying the skin (panniculitis). This form of panniculitis is called lupus profundus.
Mucosal DLE is the name given to the lesions that occasionally occur in the mucus membranes of the mouth, nose, and eyes
Palmar-plantar DLE is the name given to the lesions that occasionally occur on the hands and feet.
Subacute Cutaneous LE (SCLE)
Two clinical forms of SCLE lesions
1. The papulosquamous variety of SCLE is characterized by red (erythematous) plaques-elevated areas of scaly skin with distinct margins.
* This form of SCLE can resemble psoriasis.
* These lesions appear most commonly on the sun-exposed areas of the arms, shoulders, neck and trunk, with the face being affected less frequently.
2. The other form of SCLE consists of red annular (ring-shaped) lesions occurring on the same parts of the body.
* Both forms of SCLE are characteristically very photosensitive. This means they get worse when exposed to sunlight or artificial sources of ultraviolet light.
* Unlike DLE, SCLE lesions do not scar.
* However, they can produce areas of light or dark skin discoloration.
* As with other forms of cutaneous LE, SCLE lesions usually do not itch.
* Occasionally, other forms of LE skin disease-such as DLE and ACLE-can develop in SCLE patients. In these cases, ACLE could be a warning sign for the development of a more severe form of SLE.
* Approximately 50 percent of patients with SCLE will have a diagnosis of SLE.
* However, more severe forms of SLE are quite unusual and occur in less than 19 percent of SCLE cases. These cases will be characterized by:
o disease in the kidneys
o disease in the brain
o disease in other vital organs
* Ro/SS-A autoantibodies are found in the blood of approximately 70 percent of SCLE patients. This is the same autoantibody that is produced by patients with Sjogren's syndrome.
* The skin lesions seen in infants with neonatal LE are similar to SCLE lesions.
* The neonatal LE syndrome results when infants are born to mothers who have Ro/SS-A autoantibodies in their blood during pregnancy.
* Neonatal LE skin lesions usually spontaneously disappear by six months of age.
* A very 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).
Recent studies indicate that cold, damp weather can precipitate a peculiar form of cutaneous lupus erythematosus, called pernio, in anti Ro/SS-A antibody positive patients. This form of cutaneous lupus is characterized by a purplish dermatitis involving the fingers, ears and nose. Thus ultraviolet light as well as cold damp weather are two physical factors capable of precipitating cutaneous lupus lesions in patients with these autoantibodies.
In addition, various medications have been implicated in the induction of SCLE lesions in anti Ro/SS-A antibody positive patients. These drugs include antidiuretics (hydrochlorothiazide, spironolactone), antifungal agents (griseofulvin, terbenifine), calcium channel blockers (nefidipine, diltiazem), angiotensin converting enzyme inhibitors (Captopril), lipid lowering drugs, or statins (pravastatin, simvastatin), non-steroidal anti-inflammary drugs, or NSAIDs (piroxicam, naproxen), tumor necrosis factor inhibitors, and antimetabolites (docetaxel, paclitaxel). Once induced, the SLCE lesions respond to discontinuance of the offending agent plus the addition of traditional anti-cutaneous lupus therapy (i.e., antimalarials, oral steroids,, retinoids, etc.).
The pathophysiologic mechanism of drug-induced SCLE lesions in anti Ro/SS-A antibody positive patients is unknown at this time, but several of the above drugs are known photosensitizers and so could possibly induce SCLE by increasing the photosensitivity of the patients .
Acute Cutaneous LE (ACLE)
The most typical form of ACLE consists of flattened areas of red skin on the face that resemble a persistent sunburn.
Localized ACLE is seen when both cheeks and nose are involved. The redness can simulate the appearance of a butterfly.
Generalized ACLE is the more widespread form.
This redness can be seen on the arms, legs, and body, and often produces a rash-like appearance
ACLE lesions tend to be very photosensitive.
* They typically do not produce scarring, although skin discoloration can be seen.
* ACLE is often seen in people who have active, sometimes severe, SLE.
Skin Lesions In LE And Other Diseases
In general, this category of LE skin disease occurs in patients who also have SLE.
SLE patients may develop damage of the blood vessels in the skin called cutaneous vasculitis.
* Cutaneous vasculitis lesions typically appear as small red-purple spots and bumps on the lower legs (palpable purport).
* Occasionally, larger knots (nodules) and ulcers can develop.
* Vasculitis lesions can appear in the skin as hive-like or wheal-like lesions (urticarial vasculitis).
* The lesions also can appear as small red or purple lines or spots in the fingernail folds or on the tips or the fingers.
Hair Loss (Alopecia)
There are several forms of non-scarring hair loss which are not related to the presence of DLE of the scalp.
* SLE patients who have been severely ill with their disease may develop a temporary pattern of hair loss which is replaced by new hair growth. This condition is known as telogen effluvium.
* A severe flare of SLE 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."
Additional Forms of Skin Disease Seen in LE as Well as Other Diseases
There are a number of other forms of skin disease in this category, such as
* calcinosis (calcium deposits in the skin),
* rheumatoid nodules,
* Raynaud's phenomenon, and
* livedo reticularis.
Photosensitivity is a common feature of both cutaneous LE and SLE. The overwhelming majority of LE skin lesions occur on sun-exposed areas.
* Approximately 40-70 percent of people with LE will note that their cutaneous and/or systemic disease is aggravated by sun exposure.
* SCLE lesions are somewhat more likely to be worsened by sunlight exposure than DLE lesions.
* It is the sunburning ultraviolet B (UV-B) rays in sunlight that are particularly bad for LE patients.
* Longer wavelength ultraviolet A (UV-A) rays can also aggravate cutaneous LE, especially SCLE. As an example, a standard pane of window glass blocks UV-B rays but allows UV-A rays to pass through.
Sun protection is extremely important for people with both cutaneous LE and SLE.
1. Avoid prolonged periods of exposure to sunlight, especially between the hours of 10 a.m. and 3 p.m. when the sun is at its brightest. (Use this rule: if your shadow is as long as you are tall, it's okay to be outside).
2. Avoid exposure to reflected sunlight around highly reflective surfaces, such as sand, water, and snow.
3. Avoid artificial sources of ultraviolet rays, such as being near unshielded fluorescent lighting tubes.
4. Ensure physical protection from sunlight by using wide-brimmed hats and umbrellas.
* Tightly-woven, light-weight clothing can provide significant sun protection (specially-designed sun protective clothing is available commercially).
* The regular use of broad-spectrum sunscreens with a sun protective factor (SPF) rating of at least 15 is strongly recommended.
* The broadest protection against both UV-A and UV-B is in sunscreens that contain avobenzone (Parsol 1789), titanium dioxide, and/or zinc oxide.
Treatment of LE Lesions
* Treatment of all forms of LE skin disease begins with the use of sunscreens.
* ACLE is usually treated with systemic drugs such as prednisone to suppress accompanying SLE symptoms.
* DLE and SCLE skin lesions can be treated with the application of steroid creams, ointments, gels, and solutions.
* In addition, individual lesions can be covered with steroid-impregnated tape or injected with a steroid solution.
More widespread LE skin lesions and lesions that do not respond to the above local measures can be treated with systemic antimalarial drugs such as hydroxychloroquine capsules (brand names: Plaquenil, Quineprox).
* This drug is given by mouth, alone or in combination with quinacrine capsules and/or a short burst of steroids (prednisone).
* In stubborn cases it will be necessary to substitute chloroquine (brand name: Aralen) for hydroxychloroquine.
o Cutaneous LE lesions respond better to antimalarial therapy when the patient is not smoking cigarettes.
Other oral drugs that can be of benefit in resistant cutaneous LE cases include:
o isotretinoin [Accutane]
o etretinate [Tegison]
o acitretin [Soriatane]
* diaminodiphenylsulfone (Dapsone)
* gold (Auronofin)
* clofazimine (Lamprene)
Occasionally, stronger immunosuppressive drugs might be required to control potentially disabling cases of LE skin disease, such as:
o azathioprine (Imuran)
o cyclophosphamide (Cytoxan)
o Cyclosporine (Neoral).
Dealing With The Effects Of LE Lesions
* When properly blended and applied, corrective camouflage cosmetics can temporarily mask the appearance of the skin discoloration and scarring that can result from cutaneous LE lesions.
* Plastic surgery techniques to correct scarring from cutaneous LE can be somewhat risky. However, such techniques may be considered while the patient is under active treatment with drugs such as antimalarials. These will prevent the aggravation of skin disease that can occur in people with LE who also have any form of skin injury, including surgical manipulation.
For specific information regarding the treatment of various skin manifestations of LE, as well as the proper selection and use of sunscreens, consult your dermatologist or your local chapter of the Lupus Foundation of America.
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