Background/Purpose: Localized scleroderma (LS) can affect patients’ physical function and psychosocial well-being, but, published studies of the impact of pediatric LS on health-related quality of life (HRQoL) show mixed results (Table 1). Present studies are limited by small sample sizes and cross-sectional study designs. Furthermore, there is no current consensus on which HRQoL domains are most relevant in LS, resulting in use of generic HRQOL instruments which may not capture all HRQOL areas of interest. The goal of this study was to examine the domains captured by existing tools and to qualitatively identify new, potentially important domains of HRQoL impact through focus groups with pediatric LS patients and their parents.

Methods: Domains of LS HRQoL impact were identified in two ways: (1) review of extant literature using generic and dermatology-specific HRQoL instruments and 2) focus groups with LS patients and their parents. Three patient and 3 parent focus groups were conducted with 3-5 patients in each patient group and 4-7 parents per group. Groups were divided by patient age range (8 to 10 yo, 11 to 13 yo, 14 years and older).

Results: Table 1 summarizes generic HRQoL instruments available in the literature used with LS patients and the domains they measure. Skin symptoms and joint/muscle pain are the most well studied symptoms in pediatric LS, while effects on body image and peer relationships are less well-characterized. Seven domains were ultimately identified as potentially important contributors to HRQoL in LS (Table 2). Physical functioning is often studied but focus groups revealed effects on fine motor activity not readily captured by existing instruments (Table 2).  Side effects from treatment with systemic medications are also not currently well studied in LS, although patients and parents indicate they are bothersome (Table 2). 

Conclusion: LS patients experience adverse HRQoL impact across multiple physical and psychosocial domains, as corroborated by LS patients themselves. Existing generic HRQoL instruments do not assess all of these domains fully and underexplore domains such as treatment effects.  The literature review and focus groups yielded candidate domain for LS HRQoL measurement, but future research is needed to assess how these domains can be quantified effectively. Development of an LS-specific HRQoL tool may be indicated. Table 1: Current domains captured by existing quality of life instruments used with pediatric LS patients.

DLQI = Dermatology Life Quality Index; CDLQI = Children’s Dermatology Life Quality Index; CHAQ = Children’s Health Assessment Questionnaire, CHQ = Childhood Health Questionnaire; CQOL = Child Quality of Life Questionnaire; KINDL = German generic quality of life tool for children, ISDL = The Impact of Chronic Skin Disease on Daily Life. ¹Baildam et al. (2011). Influence of childhood scleroderma on physical function and quality of life. J Rheumatol, 38(1), 167-73. ²Klimas et al. (2014). Health-related quality of life in morphea. Br J of Dermatol, 172, 1329-37. ³Condie et al. (2014). Comparison of outcomes in adults with pediatric-onset morphea and those with adult-onset morphea. Arthritis Rheumatol, 66(12), 3496-505. ⁴Das et al. (2014). Correlates of self-reported quality of life in adults and children with morphea. J Am Acad Dermatol, 70(5), 904-10. ⁵Szramka-Pawlek et al. (2013). Health-related quality of life, optimism, and coping strategies in persons suffering from localized scleroderma. Psychol Health Med, 18(6), 654-63. ⁶Ennis et al. (2012). Children’s and parents’ beliefs about childhood onset scleroderma are influenced by child age and physical function impairment. Rheumatol, 51(7), 1331-3. ⁷Saxton-Daniels & Jacobe. (2010). An evaluation of long-term outcomes in adults with pediatric-onset morphea. Arch Dermatol, 146(9), 1044-5. ⁸Orezechowski et al. (2009). Health-related quality of life in children and adolescents with juvenile localized scleroderma. Rheumatol, 48(6), 670-2. ⁹Kroft et al. (2009). Psychological distress in patients with morphea and eosinophilic fasciitis. Arch Dermatol, 145(8), 1017-22. ¹⁰Kroft et al. (2008). Physical burden of symptoms in patients with localized scleroderma and eosinophilic faciitis. Arch Dermatol, 144(10), 1394-1395. ¹¹Uziel et al. (2000). Children with morphea have normal self-perception. J Pediatr, 137(5), 727-30. Table 2: Identified domains of health related quality of life that are impacted by pediatric localized scleroderma (LS) and corroborating quotations from LS patients and their parents.