Cutaneous Lupus Erythematosus in Skin of Color

April 2015 | Volume 14 | Issue 4 | Original Article | 343 | Copyright © 2015

Wallace Nozile MS, Cheri N. Adgerson MD, and George F. Cohen MD

Department of Dermatology, University of Florida, Gainesville, FL

Abstract

Cutaneous Lupus Erythematosus (CLE) is a common manifestation in patients with Systemic Lupus Erythematosus. In a significant population of patients, CLE is the predominant feature and, in some cases, patients suffer from cutaneous disease alone. Chronic Cutaneous Lupus Erythematosus (CCLE) is a scarring subtype, more prevalent in blacks. Patients with skin of color may pose a challenge to physicians due to exaggerated cutaneous findings and increased risk of post-inflammatory hyperpigmentation and hypertrophic scarring. With the demographics of the United States rapidly shifting towards a greater population of non-Caucasian racial and ethnic groups, it is imperative that we expand on the limited research into molecular variation, clinical presentation, and therapeutic efficacy in CLE. The purpose of this review is to bring attention to the unique and severe aspects of CLE in persons of color, which calls for early and aggressive treatment.

J Drugs Dermatol. 2015;14(4):343-349.

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INTRODUCTION

Systemic Lupus Erythematosus (SLE) is an autoimmune connective tissue disease that affects various organs of the body. In 1982, the American College of Rheumatology (ACR) revised the criteria for the diagnosis of Systemic Lupus Erythematosus (Table 1). Although the criteria were originally constructed for the purpose of research, meeting at least 4 of the 11 criteria produces a sensitivity of 88% and a specificity of 79% for SLE. Therefore these criteria are commonly used as a standard for diagnosis.1 Cutaneous Lupus Erythematosus (CLE) is the second most common finding in SLE. Cutaneous Lupus Erythematosus may also present independent of systemic involvement. The majority of patients with CLE have disease confined to the skin, while some eventually progress to systemic disease.2

Of all skin manifestation, the malar rash is most commonly associated with Cutaneous Lupus. However, there are many forms of presentation and over 75% of those suffering from SLE experience these lesions at some point during their disease.3 An inflammatory process involving cytokines, chemokines, and adhesion molecules produces the skin lesions demonstrated in CLE. These inflammatory molecules, along with T cells, B cells, and mediators of vascular change, are induced via Ultraviolet Radiation (UVR), drugs, viruses, cigarette smoke, and other triggers.2 Certain groups of patients with CLE have been observed to carry specific HLA haplotypes and specific complement deficiencies have also been linked to various subtypes of CLE 1. However, genetic factors alone cannot differentiate between the subtypes reliably.

CLE is organized into subtypes (Table 2), which differ by clinical presentation, histology and association with certain genetic factors. Dermatologists must be astute in recognizing the lesions as they may present differently in various skin types. Early detection may halt clinical progression and decrease risk of long term sequelae, especially in patients of darker skin types.

Subtypes of CLE

Acute Cutaneous Lupus Erythematosus (ACLE)

ACLE often presents with the classic malar rash, and is found in approximately half of patients suffering from SLE. This butterfly rash is a symmetric erythema that spans from cheek to cheek, passing over the nasal bridge (Figure 1). The rash may spread further laterally, involving the ears. It may also spread vertically towards the forehead or upper neck. Significant facial edema can occur. The nasolabial fold is characteristically spared due to its relatively sun-protected location.3 The sparing of this area further supports the theory that sun damage plays a role in initiating the inflammatory process resulting in the skin lesions. The butterfly rash of ACLE typically heals without scarring. Disseminated or generalized ACLE may appear as a maculo-urticarial or papular eruption, on the extensor surface of the arm and hand, sparing the knuckles.5 Similar lesions can also appear on the trunk, varying in distribution among individuals. Cheilitis, mucosal changes, digital ulcers and pitting scars may also be evident. These findings are not specific for ACLE and maybe found more commonly in other rheumatic diseases such as Dermatomyositis.3

Classic skin lesions, confirmatory serologic findings, signs and symptoms common of SLE render skin biopsy unnecessary in most cases.5 When biopsy is obtained, histologic findings in ACLE may be subtle compared to other subtypes. There is an interface dermatitis with vacuolization of the dermal-epidermal junction (DEJ), a relatively sparse superficial, lymphocytic

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