Cutaneous lupus erythematosus: diagnosis and treatment

Abstract

Cutaneous lupus erythematosus (CLE) encompasses a wide range of dermatologic manifestations, which may or may not be associated with the development of systemic disease. Cutaneous lupus is divided into several sub-types, including acute CLE (ACLE), sub-acute CLE (SCLE) and chronic CLE (CCLE). CCLE includes discoid lupus erythematosus (DLE), LE profundus (LEP), chilblain cutaneous lupus and lupus tumidus. The diagnosis of these diseases requires proper classification of the sub-type, through a combination of physical examination, laboratory studies, histology, antibody serology and occasionally direct immunofluorescence, while ensuring to exclude systemic disease. The treatment of cutaneous lupus consists of patient education on proper sun protection along with appropriate topical and systemic agents. Systemic agents are indicated in cases of widespread, scarring or treatment-refractory disease. In this chapter, we discuss issues in classification and diagnosis of the various sub-types of CLE, as well as provide an update on therapeutic management.

Keywords: Acute cutaneous lupus erythematosus; Chronic cutaneous lupus erythematosus; Cutaneous lupus erythematosus; Diagnosis; Discoid lupus erythematosus; Lupus erythematosus profundus; Lupus erythematosus tumidus; Sub-acute cutaneous lupus erythematosus; Systemic lupus erythematosus; Treatment.

Fig. 1

Algorithm for cutaneous lupus erythematosus treatment. Localized disease is initially treated with topical agents (either corticosteroids (CS) or calcineurin inhibitors (CI)). Hydroxychloroquine (HCQ) is also often used, depending on the site or if there is scarring disease. Widespread or scarring disease treatment starts with topicals and HCQ. If this fails, quinacrine is added to HCQ. If this regimen fails, a switch to chloroquine (CQ) can be made, while continuing quinacrine. If this fails, other options include mycophenolate mofetil (MM) or mycophenolate sodium (MS), azathioprine, dapsone, retinoids and thalidomide can be considered. In the case of failure of these agents, experimental therapy can be considered.