Published on 19/03/2015 by admin
Filed under Dermatology
Last modified 19/03/2015
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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
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.
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.
Autoantibody studies
Indicators of systemic disease
Walling HW, Sontheimer RD. Am J Clin Dermatol 2009; 10: 365–81.
This article reviews the three recognized subtypes of cutaneous lupus erythematosus, including the acute, subacute, and chronic forms, as well as the non-specific cutaneous manifestations of the disease. Diagnostic strategies which encompass histopathology, immunopathology, serology and other laboratory studies are discussed.
Treatment of Skin Disease Comprehensive Therapeutic Strategies 4e
