Abstract
Localized scleroderma (LoS) is a chronic fibrosing disorder of the skin and, less commonly, subcutaneous tissues. As the disease causes subjective symptoms, cosmetic defects, and, at times, functional disability, subjects with LoS experience deterioration of their health-related quality of life (HRQoL). The influence of disease activity/severity and damage status on HRQoL measures in patients with LoS is scarcely known. Physician-reported measures (modified LoS skin severity index, LoS skin damage index, physician global assessments of the disease activity/severity and damage) and patient-derived measures (patient global assessments of the disease activity/severity and damage) were obtained in adult LoS patients. Their HRQoL was measured with Skindex-29 and Short Form-36. The patients’ assessments of disease activity/severity and damage in LoS differed from the assessments by the physicians. The patients’ predominant concerns centered on LoS-related damage, whereas the physicians’ concerns focused on features of disease activity. Visual analogue scales bore some relation to the HRQoL, and they seem to be important in a holistic approach to the patient and should not be omitted in LoS evaluation.
Highlights
Localized scleroderma (LoS) is an autoimmune connective tissue disorder characterized by inflammation and fibrosis of the skin and, less commonly, deeper tissues in the affected area
Two patients had the disease onset at the age of four and five years old, and after a long period of quiescence their LoS relapsed when they were at the age of 31 and 22 years old, respectively
Most patients (35 of 40, 87.5%) had the active disease, of which six patients were experiencing a first and three patients a second relapse, but the rest were in their first episode of the disease activity
Summary
Localized scleroderma (LoS) is an autoimmune connective tissue disorder characterized by inflammation and fibrosis of the skin and, less commonly, deeper tissues (subcutaneous fat, fascia, muscles, and occasionally even bones) in the affected area. This is a rare disease, with two peak incidence periods—between the ages of 7 and 10 and between 40 and 50 with female preponderance (3:1–4:1) [1]. Neurological symptoms (e.g., seizures/epilepsy, headaches/migraines, stroke) and/or eye conditions (such as uveitis, xerophthalmia, papilledema, episcleritis, glaucoma, cataract) have been reported in some LoS cases in the head or face [4,5] The frequency of these manifestations differs depending on age at disease onset and is highest in childhood-onset LoS [1,4,5]. HRQoL may be affected by disease activity/severity and damage status, but their influence from the perspective of patients and physicians is scarcely known
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