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

Abstract

ObjectiveThe 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 literature published between 2000 and 2020 in PubMed, EMBASE, the Cochrane Skin Group Specialized Register, the Cochrane Central Register of Controlled Trials, and the Cumulative Index to Nursing and Allied Health Literature using the search terms “scleroderma, localized,” “Morphea,” “anxiety,” “depression,” “resilience,” “social stigma,” “quality of life,” “mood,” or “stress” and limited the search to pediatric patients and English language. Patient demographics, characteristics of JLS, and comorbidities were extracted. The outcomes included measures of health‐related quality of life (HRQoL), psychosocial functioning, evaluation of self‐perception, and the 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 little to no effect on HRQoL. One study used the Health‐Related Quality of Life in Children and Adolescents Questionnaire and did not find differences between children with JLS or 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 patients with JLS. These emotions were associated with skin symptoms (pain, itch, and tightness), physical limitations, and treatment burden.ConclusionOverall, 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 the HRQoL evaluation of this population.