Abstract and Introduction
Abstract
Background Studies reporting the incidence of isolated cutaneous lupus erythematosus (CLE) are rare.
Objectives To examine in a population-based cohort study the incidence of CLE and its subsets in Sweden. The short-term probability of receiving an additional diagnosis of systemic lupus erythematosus (SLE) is also assessed.
Methods A population-based open cohort study including all patients with CLE [International Classification of Diseases (ICD) code, ICD-10: L93] in Sweden, 2005–2007. Patients (n = 1088) were identified in the Swedish National Patient Register.
Results The incidence of CLE was 4·0/100 000; the female/male ratio was 3 : 1. Mean age at disease onset was 54 years. The most common subset was discoid lupus erythematosus (DLE) (80%, n =868). A quarter of the patients (24%, n = 260) were already diagnosed with SLE at the time they were diagnosed with CLE. During the whole observation period (2005–2007), an additional 18% (n = 107) were diagnosed with SLE, the probability of receiving an additional SLE diagnosis being highest for the subacute CLE (SCLE) subset.
Conclusions This is the first nationwide epidemiological study on CLE. We found the incidence of CLE to be about equal to that of SLE, and found a higher short-term probability for receiving an additional diagnosis with SLE than previously described for CLE. Subsets other than DLE and SCLE were rarely reported in our system; an update of the ICD codes for this diagnostic group could increase reporting of these more unusual cases. Our study clarifies that monitoring and follow-up are called for in this patient group due to the risk for SLE, and underscores the need for clear criteria for risk assessment in the large group of patients with CLE who also fulfil criteria for SLE.
Introduction
Lupus erythematosus (LE) is a heterogeneous disease with a wide spectrum of presenting symptoms, from localized cutaneous LE (CLE) to severe disseminated disease in systemic LE (SLE). CLE is a chronic skin disease with unknown cause in which genetic, environmental and hormonal factors are regarded as important.
The several different clinical presentations of CLE have been subdivided into acute CLE, subacute CLE (SCLE) and chronic CLE of which the most common manifestation is discoid LE (DLE). Other more rare disease subsets are lupus panniculitis (lupus profundus), LE tumidus (LET) and neonatal LE. The diagnosis relies on histopathological, serological and clinical criteria. The subsets have distinct clinical features and are described to differ in clinical course and prognosis.
The present criteria for SLE diagnosis include three cutaneous lesions and patients with mainly cutaneous disease can thus frequently fulfil four or more criteria for SLE diagnosis.
Large-scale, population-based epidemiological studies reporting the incidence of isolated CLE are rare. Furthermore, there are very few reliable figures on the age- and gender-specific distribution of the incidence of CLE.
In this study we used the Swedish National Patient Registry (NPR) [reporting diagnoses based on the International Classification of Diseases (ICD) codes classified by the World Health Organization] to estimate the age- and gender-specific incidence of CLE and its subsets in Sweden, as well as the probability for receiving a SLE diagnosis.