Lupus Erythematosus - Patches Dominate Disease

Diagnostic Hallmarks

1. Distribution- face, neck and sun-exposed areas of the upper trunk and arms
2. Sunlight sensitivity

Clinical Presentation

Lupus erythematosus (LE) is a disease that has a very broad spectrum of clinical symptoms and signs. The spectrum is continuous, but it is convenient to consider four points on the spectrum as if they were four separate conditions.

Only the discoid-type skin lesions possess all of the characteristics of papulosquamous disease. The skin lesions that occur in patients with systemic disease lack one or more papulosquamous characteristics and thus overlap morphologically with diseases of the vascular reaction and eczematous disease groups.

The lesions in all types of LE are, in general, correlated with sunlight exposure. They are primarily found on the sun-exposed portions of the body, and in many cases the patient will have noted the development of new lesions (or the worsening of old lesions) following one or more episodes of sunlight exposure. Nevertheless, a specific history of photosensitivity is often lacking.

The patches and plaques of LE are generally asymptomatic, but a sensation of swelling and burning is sometimes described by patients with the lesions of systemic LE.

The diagnosis of LE involving the skin is often possible on the basis of clinical examination. Confirmation should be obtained through biopsy, however. Biopsy of skin lesions for direct immunofluorescence (the lupus band test) is particularly helpful, since it regularly reveals the deposition of complement and immunoglobulin at the dermal-epidermal junction. Similar deposits can also be identified in the nonlesional skin in about 70% or 80% of those patients who have systemic disease. Serologic tests for fluorescent antinuclear antibodies (FANA) are also useful in the diagnosis of LE.

Discoid Lupus Erythematosus

The skin lesions of discoid LE consist of sharply marginated, erythematous plaques 1 to 4 cm in diameter. Large lesions are often annular with a thin erythematous scaling border surrounding a white, scarred center. Smaller lesions are solid (as opposed to annular) that-topped papules and plaques diffusely covered with scale. lesions of discoid LE occur anywhere on the face but are most often found on the lateral cheeks, particularly at the Lawline. The distribution, although usually bilateral, is often not symmetrical. Lesions in the scalp occur as sharply localized patches of hair loss . Gray-white plaques are sometimes found on the lips and oral mucous membranes. Discoid LE occurs with approximately equal frequency in men and women. The disease develops at any point from childhood to late adult life . Systemic symptoms and signs are almost always absent in patients with discoid lesions. The incidence in men and women is almost equal. Antinuclear antibodies as determined by fluorescent antinuclear antibody (FANA) tests are usually negative.

Disseminated Discoid Lupus Erythematosus

There is, however, less tendency for central clearing and for scarring. Moreover, the distribution pattern is more extensive; lesions are found on the sun-exposed surfaces of the arms and hands as well as on the face. Scarring alopecia is not often present. Ten percent to 20% of patients with this type of LE will have a positive F ANA test.

Subacute Lupus Erythematosus

Often, the term subacute cutaneous is used for this type of disease. Two types of lesions may occur. The first consists of lesions that are annular plaques 2 to 10 cm in diameter. The ring of the annulus is 3 to 5 mIll wide and has little or no scale. The central portion appears as normal skin. Coalescence of these lesions to form larger plaques with a gyrate configuration sometimes occurs . The second type consists of solid plaques that resemble psoriasis. Differentiation from psoriasis is possible because the margination may be a bit less distinct, the scale size is smaller, and there is no evidence of the Koebner phenomenon. Both types of subacute LE are distributed primarily on the upper trunk and lateral arms; the face is usually spared. Patients with subacute LE usually have a positive FANA test; more specifically, Ro (SSA) antibodies are regularly present.

Acute Systemic Lupus Erythematosus

The cutaneous hallmark of acute systemic LE is the presence of symmetrical, poorly marginated, erythematous plaques on the upper malar prominences. The bridge of the nose may also be involved. Scale formation in this so-called butterfly eruption is minimal, and the plaque is usually somewhat edematous. When lesions occur other than on the face, there is a marked tendency for coalescence, as opposed to the smaller discrete lesions of discoid LE. Hair loss, when it occurs, is diffuse rather than localized. Mucous membrane lesions occur in about 25% of patients, they are identical with those seen in discoid LE .

Both sides of the hands are regularly involved. Small patches of erythema are located on the dorsal surface of the phalanges, but the area over the knuckles is spared. Reddening and telangiectasia are frequently present in a narrow band at the posterior nail folds. The palmar surfaces of the hands are often violaceous. This color change is particularly notable over the tips of the fingers and on the thenar and hypothenar eminences. Small, bright red, blanching macules or pinpoint violaceous vasculitic lesions may be superimposed against these duskier color changes. Women with this condition outnumber men with this condition by a considerable margin.

Course and Prognosis

The course and prognosis of LE correlate rather well with various types of cutaneous lesions. Patients with discoid lesions confined to the face may have a few minor laboratory abnormalities but rarely, if ever, have symptoms and signs of systemic disease. Moreover, 95% of such patients will have a normal life span with only cutaneous morbidity as a manifestation of their disease.

Patients with disseminated discoid skin lesions usually have a number of minor laboratory abnormalities, but they, too, rarely develop significant systemic disease.

Patients with lesions of subacute LE often have fever and arthralgia, but cardiac, central nervous system, and renal involvement are usually mild or absent. Patients with lesions of acute LE are highly likely to have serious systemic symptoms and signs.

The lesions of discoid LE heal with scarring and sometimes “burn out” altogether after 10 to 20 years of activity. The lesions of subacute and acute LE heal without scarring. These latter lesions tend to mirror the activity of the underlying systemic disease; i.e., they fade during periods of remission and reappear during exacerbations.

Pathogenesis

The cause of skin lesions in LE is not known. In most instances, sunlight seems to play an important precipitating role. Exposure to the 280- to 320-nm wavelengths of ultraviolet light (the UVB, or sunburn, spectrum) presumably leads to DNA damage in epithelial cells. It is hypothesized that this modified DNA then acts as a new antigen that stimulates the production of “autoimmune” antibodies. The production of these antibodies by B cells may be enhanced by a reduction in suppressor T cells. Antibodies, once formed, are deposited along with complement in the skin and other organs. Genetic factors, as manifest by the frequency of familial cases and the presence of certain HLA patterns, are undoubtedly important. Since the more serious forms of LE occur primarily in women, it is suspected that the presence of estrogens (or absence of androgens) may playa role.

Therapy

All patients should be protected from ultraviolet light irradiation in the UVB (sunburn) spectrum. This can be accomplished rather well by regular application of sunscreens with a high sun protective factor (SPF) . In addition, protective clothing and a change in lifestyle that moves outdoor activities to the beginning and end of the day are recommended.

The cutaneous lesions of LE resolve with steroid treatment. Discoid lesions respond inconsistently to topical steroids but do improve with intralesional injections of triamcinolone. The lesions of subacute and acute LE clear if associated systemic disease is treated with systemic steroids. The oral administration of hydroxychloroquine (Plaquenil) in a daily dose of 200 to 400 mg is very helpful in the treatment of all types of lesions






 

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