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Clinical and demographic characteristics of patients with scleroderma

Abstract:

Introduction: Scleroderma is a term used to refer to a group of fibrosing disorders affecting the skin. Two types of scleroderma can be differentiated: systemic sclerosis (SS), with visceral involvement, and localised scleroderma or morphea, confined to the skin and underlying tissues. Few studies have evaluated the characteristics of these patients. The aim of this study was to analyse demographic and clinical features of patients with a diagnosis of scleroderma. Material and methods: An analysis was performed on the data from adult patients with a diagnosis of scleroderma and followed-up in a single speciality clinic in a tertiary hospital. Results: The study included 100 patients (80 with SS and 20 with morphea). The median age of onset was 45 years, and women were more frequently affected than men. Patients were classified according to the subtype of scleroderma. Prevalence of concomitant autoimmune diseases (28% patients) and lichen sclerosus (5%) were also analysed. Another classification is proposed by us for patients with morphea according to the distribution of lesions: isomorphic morphea (lesions on areas of skin friction) and non-isomorphic morphea (other locations). Patients with isomorphic morphea were more predominantly female, with a higher prevalence of overweight and obesity, and a higher association with lichen sclerosus. Patients with non-isomorphic morphea required systemic treatment less frequently. Conclusions: The present study suggests that a classification for patients with morphea according to distribution patterns could differentiate two subtypes of patients that seem to have differences in disease course.