Cutaneous Lupus Erythematosus: An Update on Pathogenesis, Diagnosis and Treatment

Emily Z. Hejazi; Victoria P. Werth

Disclosures

Am J Clin Dermatol. 2016;17(2):135-146. 

In This Article

Abstract and Introduction

Abstract

Cutaneous lupus erythematosus (CLE) includes a broad range of dermatologic manifestations, which may or may not be associated with systemic disease. Recent studies in this area continue to shape our understanding of this disease and treatment options. Epidemiologic studies have found an incidence of CLE of 4.30 per 100,000, which approaches similar analysis for systemic lupus erythematosus (SLE). Although there have been extensive efforts to define SLE, the classification of CLE and its subgroups remains a challenge. Currently, diagnosis relies on clinical and laboratory findings as well as skin histology. The Cutaneous Lupus Area and Severity Index™ (CLASI™) is a validated measure of disease activity and damage. CLE pathogenesis is multifactorial and includes genetic contributions as well as effects of ultraviolet (UV) light. Immune dysregulation and aberrant cell signaling pathways through cytokine cascades are also implicated. Patient education and avoidance of triggers are key to disease prevention. Antimalarials and topical steroids continue to be the standard of care; however, immunosuppressants, thalidomide analogs and monoclonal antibodies are possible systemic therapies for the treatment of recalcitrant disease.

Introduction

Lupus erythematosus (LE) is an autoimmune disease with a variety of clinical manifestations ranging from multi-organ system involvement [systemic lupus erythematosus (SLE)] to limited cutaneous disease. Dermatologic findings are often an early sign of LE and are a manifestation of the disease in a majority of patients. While LE pathogenesis is not fully understood, it is a multifactorial disorder with genetic and environmental contributions, leading to immune activation. LE associated skin lesions show varied expression, leading to the broad classification as LE specific and LE nonspecific.[1] The latter include vascular changes such as livedo reticularis, periungual telangiectasias, Raynaud's phenomenon, erythema multiforme, and calcinosis cutis, findings usually associated with SLE but also seen with other disorders. LE-specific lesions refer to the subtypes of cutaneous lupus erythematosus (CLE) that are grouped on the basis of histology, duration of lesions, laboratory abnormalities, and clinical findings. These are grouped into acute cutaneous LE (ACLE), subacute cutaneous LE (SCLE) (Fig. 1), and chronic cutaneous LE (CCLE), which includes discoid LE (DLE) (Fig. 2) and LE profundus (LEP), chilblain LE (CHLE), and LE tumidus (LET).

Figure 1.

Subacute cutaneous lupus erythematous lesions with papulosquamous plaques on the upper back, some of which have associated white scale. Note the photodistribution

Figure 2.

Discoid lupus erythematosus lesion with erythema and hyperkeratosis with hypopigmentation and hyperpigmentation at the periphery

CLE may result in significant disfigurement and discomfort, leading to poor quality of life. Avoidance of triggers such as significant sun exposure is key to preventing symptoms. Treatment with topical steroids may be sufficient for mild disease. Oral antimalarials are the standard of care for more severe presentations. If these do not work or if the disease is more aggressive, the use of other immunomodulatory and immunosuppressant therapies, as well as biologic therapies, are possible alternatives.

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