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Management of refractory discoid lupus and subacute cutaneous lupus

Jennie Clarke, MD
Section Editor
Jeffrey Callen, MD, FACP, FAAD
Deputy Editor
Abena O Ofori, MD


Discoid lupus erythematosus (DLE) and subacute cutaneous lupus erythematosus (SCLE) are variants of cutaneous lupus erythematosus (LE) that may occur independently or as manifestations of systemic lupus erythematosus. DLE most commonly occurs on the head and is characterized by well-defined inflammatory plaques that evolve into atrophic, disfiguring scars (picture 1A-B). SCLE typically presents with erythematous, scaly papules or annular plaques on the neck, upper trunk, and arms (picture 2A-B). The symptomatic and disfiguring nature of DLE and SCLE underlies the need for appropriate treatment.

Most patients with DLE or SCLE will respond to conservative therapy with topical agents and antimalarial drugs; however, when the response to these interventions is inadequate, alternative immunomodulating or immunosuppressive drugs must be utilized. The management of patients with refractory DLE and SCLE will be discussed here; the initial management of these disorders and the clinical manifestations of DLE and SCLE are reviewed elsewhere. (See "Initial management of discoid lupus and subacute cutaneous lupus" and "Overview of cutaneous lupus erythematosus".)


For most patients with cutaneous LE, strict photoprotection combined with topical or intralesional corticosteroids, topical calcineurin inhibitors, or systemic antimalarial agents results in satisfactory improvement. When disease is refractory to these interventions, other systemic agents are utilized. The adverse effects of many of these drugs limit their use to the subset of patients who fail to respond to conventional therapy.

Data on the efficacy of treatments for refractory DLE and SCLE primarily are limited to small open-label studies, retrospective reviews, case series, and case reports. The scarcity of high quality studies stems from a lack of pharmaceutical, governmental, and foundational support for large, multicenter, controlled trials in cutaneous LE, as well as the relative rarity of refractory cutaneous LE.

In addition, the historical absence of standardized objective measures of disease activity in cutaneous LE has made it difficult to systematically interpret the results of much of the published literature [1]. In 2005, the Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI) was developed and validated as a tool to assess disease activity and skin damage in cutaneous LE [2]. The CLASI is now being used in a number of clinical trials, and should improve the quality and applicability of data from studies of cutaneous LE [3]. A revised version of CLASI has also been proposed [4].


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Literature review current through: Sep 2016. | This topic last updated: Oct 22, 2015.
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