This article provides an overview of cutaneous lupus erythematosus (CLE), including classification schemes, disease subtypes, and therapy. It also describes a novel clinical outcome instrument called the Cutaneous Lupus Erythematosus Disease Area and Severity Index, which quantifies cutaneous activity and damage in CLE.
KeywordsCutaneous lupus erythematosus; disease classification; Rowell's syndrome; CLASI; clinical outcome instrument
OVERVIEW OF CUTANEOUS LUPUS ERYTHEMATOSUS Disease ClassificationCutaneous LE skin lesions have been divided into two categories based on histopathology, LE-specific (histopathology shows interface dermatitis, which is specific for LE) and LEnonspecific (no interface dermatitis, histopathology is not specific for LE) [1,2]. The diagnosis of cutaneous LE can be confirmed by the presence of LE-specific lesions, whereas LE-nonspecific lesions may be seen in several diseases and thus are not sufficient for establishing a diagnosis of cutaneous LE. LE-specific skin lesions can be further subdivided based on clinical characteristics into acute cutaneous LE (ACLE), subacute cutaneous LE (SCLE), and chronic cutaneous LE (CCLE) [2]. Table 1 [ [2][3][4] summarizes the classification of skin lesions seen in LE patients.The risk of systemic LE (SLE) is highest in ACLE and lowest in CCLE, with SCLE falling in between. In one study of 191 patients with LE-specific skin lesions, the prevalence of underlying SLE was 72% in all patients with ACLE lesions, 58% in all patients with SCLE lesions, 28% in all patients with DLE lesions (the most common type of CCLE), and 6% in all patients with localized DLE lesions (limited to the head and neck) [5]. Notably, many patients from this study had lesions from more than one clinical category (ACLE, SCLE, or CCLE), and some had lesions from all three categories. In patients with DLE lesions but no ACLE or SCLE lesions, the underlying prevalence of SLE was 15%.Corresponding author for proof and reprints: Dr. Victoria P. Werth Department of Dermatology Perelman Center for Advanced Medicine, Suite 1-330A 3400 Civic Center Boulevard Philadelphia, PA 19104 Tel. 215-823-4208, Fax 866-755- Approximately 50% of SCLE patients fulfill the criteria for SLE [5], but SLE patients with SCLE appear to have fewer organ systems involved than SLE patients without SCLE. One study that compared inpatients with both SLE and SCLE to inpatients with only SLE found an increased prevalence of central nervous system disease, renal disease, arthritis, anemia, and pleuritis in the SLE-only group [24]. Another study that compared outpatients with SCLE (some also had SLE) to outpatients with SLE alone found an increased frequency of serositis (pleuritis or pericarditis) and hematologic abnormalities (hemolytic anemia, thrombocytopenia, or leukopenia) in the SLE-only group [25].Although drug-induced DLE is very rare, numerous drugs have been reported to induce SCLE. Drug-induced SCLE resembles idiopathic SCLE both clinically (papulosquamous or annular-polycyclic photodistributed les...