Abstract

BackgroundLocalized scleroderma (LoS) affects both children and adults and is associated with permanent functional and cosmetic impairment, and reduced quality of life predominating in adults. The Localized Scleroderma Cutaneous Assessment Tool (LoSCAT) is a clinical instrument designed to measure an activity and damage of LoS. It has been validated for use with pediatric LoS patients. This study assessed the validity and reliability of the LoSCAT adapted for use in adults.MethodsBefore the initiation of the study two examiners participated in an intensive training course carried out by an expert in LoS. Appendices describing each LoSCAT domain were prepared. Features determining disease activity and damage in adult LoS patients were identified to properly evaluate the physician (Phys) and patient (Pt) global assessment (GA) of disease activity (A)/severity (S) and damage (D), which were used to assess convergent validity of the LoSCAT. Correlations of physician- and patient-derivied measures with Skindex-29 were also analysed.ResultsThe study included 40 adult LoS patients (33 females and 7 males) with different subtypes of LoS. Intra and inter-rater reliability of the LoSCAT was found to be excellent. Positive correlations were observed between the PhysGA-A, PhysGA-D, PtGA-A and the LoSCAT’s domains, while no correlations between them and the PtGA-D were found. There were no relationships between LoSCAT’s components and Skindex-29.ConclusionsDespite the LoSCAT is a reliable tool for an assessment of cutaneous lesions, additional health status instruments are necessary to a holistic approach to LoS in adults.

Highlights

  • Localized scleroderma (LoS) affects both children and adults and is associated with permanent functional and cosmetic impairment, and reduced quality of life predominating in adults

  • A few scoring systems have been proposed for this purpose: the visual analogue scale (VAS), the computerised skin score, the DIET score (D, dyspigmentation/hyperpigmentation; I, induration; E, erythema and T, telangiectasias) and the Skrzypek-Salamon et al Health and Quality of Life Outcomes (2018) 16:185

  • LoS, we evaluated the convergent validity of the Localized Scleroderma Cutaneous Assessment Tool (LoSCAT) by examining its relationship with global assessment (GA)

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Summary

Introduction

Localized scleroderma (LoS) affects both children and adults and is associated with permanent functional and cosmetic impairment, and reduced quality of life predominating in adults. The Localized Scleroderma Cutaneous Assessment Tool (LoSCAT) is a clinical instrument designed to measure an activity and damage of LoS. It has been validated for use with pediatric LoS patients. Localized scleroderma (LoS) is a chronic connective tissue disorder characterised by sclerosis of the skin and, in some cases, deeper tissues (subcutaneous fat, muscles or bones). The disease affects both children and adults, with the linear subtype predominating in children and the plaque and generalised subtypes predominating in adults [1]. A few scoring systems have been proposed for this purpose: the visual analogue scale (VAS), the computerised skin score, the DIET score (D, dyspigmentation/hyperpigmentation; I, induration; E, erythema and T, telangiectasias) and the Skrzypek-Salamon et al Health and Quality of Life Outcomes (2018) 16:185

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