Systematic Review of Health‐Related Quality of Life Impact in Juvenile Localized Scleroderma

Abstract

IntroductionThe prevalence and types of psychosocial complications of juvenile localized scleroderma (JLS), also known as morphea, an inflammatory and sclerosing disease involving the skin, fascia, muscle, and bone are poorly understood.MethodsWe performed a systematic review of PUBMED, EMBASE, Cochrane Skin Group Specialized Register, Cochrane Central Register of Controlled Trials (CENTRAL), and the Cumulative Index to Nursing and Allied Health Literature (CINAHL) between 2000 to 2020 using search terms: “scleroderma, localized” or “Morphea”, “anxiety” or “depression” or “resilience” or “social stigma” or “quality of life” or “mood” or “stress” and limited to pediatric patients and English language. Patient demographics, characteristics of JLS, and comorbidities were extracted. Outcomes included: measures of health‐related quality of life (HRQoL), psychosocial functioning, evaluation of self‐perception and treatment burden of the study population. The protocol was registered with PROSPERO (CRD42021257124). Thematic synthesis generated descriptive analysis.ResultsThirteen studies fulfilled the inclusion criteria: three retrospective cohort studies, two prospective cohort studies and eight cross‐sectional studies. A total of 690 pediatric patients with JLS were included (n=484 with linear scleroderma). Six studies used the Children's Dermatology Life Quality Index, reporting small to no‐effect in HRQoL. One study used the health‐Related Quality of Life in Children and Adolescents Questionnaire, and did not find differences between children with JLS, atopic dermatitis and healthy controls. One study used a self‐perception questionnaire that showed normal self‐worth of patients with JLS. Two studies used focus groups, both reporting elevated levels of stress, decreased self‐worth, “feeling different”, and bullying/teasing in JLS patients. These emotions were associated with skin symptoms (pain, itch, and tightness), physical limitations, and treatment burden.ConclusionsOverall, quantitative studies did not report a statistically significant impairment in HRQoL in JLS. However, qualitative studies (focus groups) reported significant psychosocial impacts related to JLS. There is a need to develop a JLS‐specific tool for HRQoL evaluation of this population.This article is protected by copyright. All rights reserved.