Discoid lupus erythematosus

Published on 19/03/2015 by admin

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Discoid lupus erythematosus

Bruce H. Thiers

Evidence Levels:  A Double-blind study  B Clinical trial ≥ 20 subjects  C Clinical trial < 20 subjects  D Series ≥ 5 subjects  E Anecdotal case reports

image

Discoid lupus erythematosus (DLE) is the most common form of chronic cutaneous lupus erythematosus (CCLE). Lesions predominate in sun-exposed areas, especially the face, scalp, upper chest, upper back, and extensor arms. Early lesions consist of sharply demarcated, erythematous, often hyperpigmented, hyperkeratotic papules and small plaques with adherent scale. The individual lesions spread peripherally, resulting in atrophy and central scarring which may be associated with alopecia, telangiectasia, and depigmentation.

Management strategy

The lesions of DLE are quite characteristic, especially in their later stages. When the diagnosis is in doubt, a skin biopsy should be performed. The histologic findings are usually diagnostic, although direct immunofluorescence examination can be obtained in questionable cases. A complete history and physical examination should be performed, looking for signs of systemic disease. Laboratory examinations to be obtained include a complete blood count with differential, erythrocyte sedimentation rate, serum chemistry profile, and urinalysis. Serum should be screened for antinuclear antibodies (ANA) and Ro(SSA)/La(SSB) antibodies. It should be emphasized that although, as mentioned above, DLE is the most common form of CCLE, most patients do not have systemic involvement. Risk factors for systemic disease include widespread skin lesions, anemia or leukopenia, and a positive ANA, especially when the titer is high. Despite the relative infrequency of internal involvement, aggressive treatment of DLE is warranted because the scarring from the disease can be devastating. The characteristic ‘carpet tack’ scale associated with lesions indicates follicular involvement, and the disease can result in permanent scarring alopecia. Moreover, the depigmentation in fully evolved lesions can be disfiguring, especially in dark-skinned individuals. The goal of therapy is to halt the inflammatory process quickly and effectively to prevent these changes. The predominance of lesions in exposed areas emphasizes the urgency for prompt effective therapy.

Patients should be counseled on the role of UV light in the provocation of skin lesions, and a program of sun avoidance and sunscreen use should be instituted. Corticosteroids, either topical or intralesional, are the cornerstone of initial therapy for patients with limited involvement. Hydroxychloroquine and other antimalarial drugs appear to afford a measure of photoprotection and are often quite effective, although their onset of action is relatively slow. Systemic retinoids are useful, especially for hyperkeratotic lesions. Dapsone, which is more commonly used for lesions of bullous lupus erythematosus, is occasionally effective for patients with DLE, as is another antibiotic, sulfasalazine. Cytotoxic agents are generally reserved for refractory cases. The role of thalidomide in the treatment of DLE is evolving. A possible place for biologic agents in the treatment of DLE has been suggested as increased knowledge is gained regarding the pathogenetic mechanisms responsible for the disease.

First-line therapies

image Sunscreens B
image Topical or intralesional corticosteroids B
image Topical immunosuppressive agents B

Effectiveness of topical calcineurin inhibitors as monotherapy or in combination with hydroxychloroquine in cutaneous lupus erythematosus.

Avgerinou G, Papafragkaki DK, Nasiopoulou A, Arapaki A, Katsambas A, Stavropoulos PG. J Eur Acad Dermatol Venereol 2012; 26: 762–7.

The macrolactam immunosuppressive agents, tacrolimus and pimecrolimus, have been reported to be effective in the topical treatment of lesions of DLE, although most reports have consisted of uncontrolled case studies. This randomized controlled trial confirms that they may provide at least temporary benefit for patients with acute, edematous, non-hyperkeratotic lesions, particularly when cutaneous atrophy, either disease- or treatment-related, is a concern. Hypertrophic lesions may not respond well to calcineurin inhibitors or to other topical therapies, presumably because of limited penetration. Topical calcineurin inhibitors can also be used in combination with systemic therapy, e.g., hydroxychloroquine.

Second-line therapies

image Antimalarial drugs B
image Systemic retinoids B

Low blood concentrations of hydroxychloroquine in patients with refractory cutaneous lupus erythematosus: a French multicenter prospective study.

Frances C, Cosnes A, Duhaut P, Zahr N, Soutou B, Ingen-Housz-Oro S, et al. Arch Dermatol 2012; 148: 479–84.

Antimalarial drugs are favored for long-term treatment of DLE and are effective in many patients for whom topical therapy alone is unsuccessful or impractical. In most patients, 6 weeks of treatment is needed before they begin to exert their effect. Hydroxychloroquine (200 mg once or twice daily) is most often used, chloroquine (250-500 mg daily) being reserved for unresponsive patients. Quinacrine was widely used in the past but has become increasingly difficult to obtain. Baseline lupus severity may predict response to hydroxychloroquine, whereas the deleterious role of cigarette smoking has likely been overstated. Treatment failure may be associated with subtherapeutic blood concentrations of the drug.

Third-line therapies

image Cytotoxic agents D
image Immune response modifying agents D
image Antibiotics D

[/level-membership-for-dermatology-category][not-level-membership-for-dermatology-category]

Discoid lupus erythematosus

Bruce H. Thiers

Evidence Levels:  A Double-blind study  B Clinical trial ≥ 20 subjects  C Clinical trial < 20 subjects  D Series ≥ 5 subjects  E Anecdotal case reports

image

Discoid lupus erythematosus (DLE) is the most common form of chronic cutaneous lupus erythematosus (CCLE). Lesions predominate in sun-exposed areas, especially the face, scalp, upper chest, upper back, and extensor arms. Early lesions consist of sharply demarcated, erythematous, often hyperpigmented, hyperkeratotic papules and small plaques with adherent scale. The individual lesions spread peripherally, resulting in atrophy and central scarring which may be associated with alopecia, telangiectasia, and depigmentation.

Management strategy

The lesions of DLE are quite characteristic, especially in their later stages. When the diagnosis is in doubt, a skin biopsy should be performed. The histologic findings are usually diagnostic, although direct immunofluorescence examination can be obtained in questionable cases. A complete history and physical examination should be performed, looking for signs of systemic disease. Laboratory examinations to be obtained include a complete blood count with differential, erythrocyte sedimentation rate, serum chemistry profile, and urinalysis. Serum should be screened for antinuclear antibodies (ANA) and Ro(SSA)/La(SSB) antibodies. It should be emphasized that although, as mentioned above, DLE is the most common form of CCLE, most patients do not have systemic involvement. Risk factors for systemic disease include widespread skin lesions, anemia or leukopenia, and a positive ANA, especially when the titer is high. Despite the relative infrequency of internal involvement, aggressive treatment of DLE is warranted because the scarring from the disease can be devastating. The characteristic ‘carpet tack’ scale associated with lesions indicates follicular involvement, and the disease can result in permanent scarring alopecia. Moreover, the depigmentation in fully evolved lesions can be disfiguring, especially in dark-skinned individuals. The goal of therapy is to halt the inflammatory process quickly and effectively to prevent these changes. The predominance of lesions in exposed areas emphasizes the urgency for prompt effective therapy.

Patients should be counseled on the role of UV light in the provocation of skin lesions, and a program of sun avoidance and sunscreen use should be instituted. Corticosteroids, either topical or intralesional, are the cornerstone of initial therapy for patients with limited involvement. Hydroxychloroquine and other antimalarial drugs appear to afford a measure of photoprotection and are often quite effective, although their onset of action is relatively slow. Systemic retinoids are useful, especially for hyperkeratotic lesions. Dapsone, which is more commonly used for lesions of bullous lupus erythematosus, is occasionally effective for patients with DLE, as is another antibiotic, sulfasalazine. Cytotoxic agents are generally reserved for refractory cases. The role of thalidomide in the treatment of DLE is evolving. A possible place for biologic agents in the treatment of DLE has been suggested as increased knowledge is gained regarding the pathogenetic mechanisms responsible for the disease.

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