Systemic sclerosis (SSc) is a chronic autoimmune disease that significantly impacts physical and psychosocial health. This study assessed skin symptoms in 126 SSc patients from a dermatological hospital Italy, using the items from the Skindex-29 for all symptoms and psychosocial variables. Multivariate logistic regression and attributable risk among the exposed (ARexp) were used for analysis. Pain (74.0%), burning (62.1%), and water irritation (52.4%) were the most reported symptoms. Older patients (≥58 years; mean age = 58.7, SD = 12.01) and those with severe disease reported higher symptom frequencies. Water irritation strongly predicted shame (OR 21.3) and frustration (OR 5.8), with ARexp indicating that alleviating water-related discomfort could reduce shame by 95.3%, frustration by 82.8%, and tiredness by 92.1%. Pain and itch were linked to depression and sleep loss. Findings highlight the significant psychosocial burden of SSc skin symptoms, emphasizing the need for targeted interventions to improve both physical and mental health outcomes.
AbeniDPicardiAPasquiniP, et al. (2002) Further evidence of the validity and reliability of the skindex-29: An Italian study on 2,242 dermatological outpatients. Dermatology204(1): 43–49.
2.
ArnoldHJFeldmanDC (1981) Social desirability response bias in self-report choice situations. Academy of Management Journal24(2): 377–385.
3.
AsanoY (2020) The pathogenesis of systemic sclerosis: An understanding based on a common pathologic cascade across multiple organs and additional organ-specific pathologies. Journal of Clinical Medicine9(9): 2687.
4.
BarbieriJSMacDonaldK (2023) Exploring discordance between patients and clinicians—understanding perceived disease severity. JAMA Dermatology159(8): 807–809.
5.
Bellando-RandoneSDel GaldoFLepriG, et al. (2021) Progression of patients with Raynaud’s phenomenon to systemic sclerosis: A five-year analysis of the European Scleroderma Trial and Research group multicentre, longitudinal registry study for very early diagnosis of systemic sclerosis (VEDOSS). The Lancet Rheumatology3(12): e834–e843.
6.
BoydKSheaSMPattersonJW (2014) The role of capsaicin in dermatology. Progress in Drug Research68: 293–306.
7.
CoiABarsottiSSantoroM, et al. (2021) Epidemiology of systemic sclerosis: A multi-database population-based study in Tuscany (Italy). Orphanet Journal of Rare Diseases16(1): 90.
8.
El AoufyKPezzuttoAPollinaA, et al. (2023) Systemic sclerosis patients experiencing mindfulness-based stress reduction program: The beneficial effect on their psychological status and quality of life. International Journal of Environmental Research and Public Health20(3): 2512.
9.
El-BaalbakiGRazykovIHudsonM, et al. (2010) Association of pruritus with quality of life and disability in systemic sclerosis. Arthritis Care & Research62(10): 1489–1495.
10.
FisherPLSalmonPHeffer-RahnP, et al. (2020) Predictors of emotional distress in people with multiple sclerosis: A systematic review of prospective studies. Journal of Affective Disorders276: 752–764.
11.
FrantzCHuscherDAvouacJ, et al. (2020) Outcomes of limited cutaneous systemic sclerosis patients: Results on more than 12,000 patients from the EUSTAR database. Autoimmunity Reviews19(2): 102452.
12.
FrechTHaysRDMaranianP, et al. (2011) Prevalence and correlates of sleep disturbance in systemic sclerosis—results from the UCLA scleroderma quality of life study. Rheumatology50(7): 1280–1287.
13.
FrechTMBaronM (2013) Understanding itch in systemic sclerosis in order to improve patient quality of life. Clinical and Experimental Rheumatology31(2 Suppl 76): 81–88.
14.
FreedmanRRIanniPWenigP (1984) Behavioral treatment of Raynaud’s phenomenon in scleroderma. Journal of Behavioral Medicine7(4): 343–353.
15.
GholizadehSFoxRSMillsSD, et al. (2017) Coping with the disfigurement of scleroderma: Facial, skin, and hand changes. In: VargaJDentonCPWigleyFM, et al. (eds) Scleroderma: From Pathogenesis to Comprehensive Management. Cham: Springer International Publishing, pp.713–721.
16.
GourierGThérénéCMazeasM, et al. (2018) Clinical characteristics of pruritus in systemic sclerosis vary according to the autoimmune subtype. Acta Dermato-Venereologica98(8): 735–741.
17.
GreenzaidJDKiritsisNRChandaniBM, et al. (2024) The psychosocial impact of immune-mediated skin diseases. In: RezaeiNYazdanpanahN (eds) PsychoNeuroImmunology: Volume 2: Interdisciplinary Approaches to Diseases. Cham: Springer Nature Switzerland, pp.601–628.
18.
GumuchianSTPeláezSDelisleVC, et al. (2016) Exploring sources of emotional distress among people living with scleroderma: A focus group study. PLoS One11(3): e0152419.
19.
HyphantisTNTsifetakiNSiafakaV, et al. (2007) The impact of psychological functioning upon systemic sclerosis patients’ quality of life. Seminars in Arthritis and Rheumatism37(2): 81–92.
20.
IBM A, NY, USA (2019) IBM SPSS statistics for windows, Version 26.0 [Software]. Armonk, NY: IBM Corp.
21.
KhannaDAllanoreYDentonCP, et al. (2020) Patient perception of disease burden in diffuse cutaneous systemic sclerosis. Journal of Scleroderma and Related Disorders5(1): 66–76.
22.
KnoblerRMoinzadehPHunzelmannN, et al. (2017) European dermatology forum s1-guideline on the diagnosis and treatment of sclerosing diseases of the skin, Part 1: Localized scleroderma, systemic sclerosis and overlap syndromes. Journal of the European Academy of Dermatology and Venereology31(9): 1401–1424.
23.
Kowal-BieleckaOFransenJAvouacJ, et al. (2017) Update of EULAR recommendations for the treatment of systemic sclerosis. Annals of the Rheumatic Diseases76(8): 1327–1339.
24.
KroftEBGroeneveldTJSeygerMM, et al. (2009) Efficacy of topical tacrolimus 0.1% in active plaque morphea: Randomized, double-blind, emollient-controlled pilot study. American Journal of Clinical Dermatology10(3): 181–187.
25.
KwakkenbosLBluyssenSJVonkMC, et al. (2011) Addressing patient health care demands in systemic sclerosis: Pre-post assessment of a psycho-educational group programme. Clinical and Experimental Rheumatology29(2 Suppl 65): S60–S65.
26.
KwakkenbosLDelisleVCFoxRS, et al. (2015) Psychosocial aspects of scleroderma. Rheumatic Disease Clinics41(3): 519–528.
27.
KwakkenbosLWillemsLMvan Den HoogenFHJ, et al. (2014) Cognitive-behavioural therapy targeting fear of progression in an interdisciplinary care program: A case study in systemic sclerosis. Journal of Clinical Psychology in Medical Settings21(4): 297–312.
28.
LeeYCFoxRSKwakkenbosL, et al. (2021) Pain levels and associated factors in the scleroderma patient-centered intervention network (SPIN) cohort: A multicentre cross-sectional study. The Lancet Rheumatology3(12): e844–e854.
29.
MeridorKBerookhimJLevyY (2022) Low dose naloxone for pruritus in systemic sclerosis: Case series and literature review. Medicine101(4): e28653.
30.
MiletteKHudsonMKörnerA, et al. (2013) Sleep disturbances in systemic sclerosis: Evidence for the role of gastrointestinal symptoms, pain and pruritus. Rheumatology52(9): 1715–1720.
31.
MiletteKThombsBDMaiorinoK, et al. (2019) Challenges and strategies for coping with scleroderma: Implications for a scleroderma-specific self-management program. Disability and Rehabilitation41(21): 2506–2515.
32.
MoinzadehPKuhrKSiegertE, et al. (2020) Older age onset of systemic sclerosis – accelerated disease progression in all disease subsets. Rheumatology59(11): 3380–3389.
33.
NakayamaATunnicliffeDJThakkarV, et al. (2016) Patients’ perspectives and experiences living with systemic sclerosis: A systematic review and thematic synthesis of qualitative studies. Journal of Rheumatology43(7): 1363–1375.
34.
NassarE-LAbdulkareemDAThombsBD (2023) Results from a living systematic review of the prevalence of mood and anxiety disorders and factors associated with symptoms in systemic sclerosis. Scientific Reports13(1): 5181.
35.
PagkopoulouEArvanitakiADaoussisD, et al. (2019) Comorbidity burden in systemic sclerosis: Beyond disease-specific complications. Rheumatology International39(9): 1507–1517.
36.
ParkEHStrandVOhYJ, et al. (2019) Health-related quality of life in systemic sclerosis compared with other rheumatic diseases: A cross-sectional study. Arthritis Research & Therapy21(1): 61.
37.
ParodisIGomezATsoiA, et al. (2023) Systematic literature review informing the EULAR recommendations for the non-pharmacological management of systemic lupus erythematosus and systemic sclerosis. RMD Open9(3): e003297.
38.
PelechasEKaltsonoudisEVoulgariPV, et al. (2023) Scleroderma. In: PelechasEKaltsonoudisEVoulgariPV, et al. (eds) Illustrated Handbook of Rheumatic and Musculo-Skeletal Diseases. Cham: Springer International Publishing, pp.257–292.
39.
PigaMChessaEMorandEF, et al. (2022) Physician global assessment international standardisation consensus in systemic lupus erythematosus: The PISCOS study. The Lancet Rheumatology4(6): e441–e449.
40.
RacineMHudsonMBaronM, et al. (2016) The impact of pain and itch on functioning and health-related quality of life in systemic sclerosis: An exploratory study. Journal of Pain and Symptom Management52(1): 43–53.
41.
RazykovILevisBHudsonM, et al. (2013) Prevalence and clinical correlates of pruritus in patients with systemic sclerosis: An updated analysis of 959 patients. Rheumatology52(11): 2056–2061.
42.
RirashFTingeyPCHardingSE, et al. (2017) Calcium channel blockers for primary and secondary Raynaud’s phenomenon. Cochrane Database of Systematic Reviews12(12): Cd000467.
43.
RomanazzoSCeccatelliSMansuetoG, et al. (2024) Well-being therapy in systemic sclerosis outpatients: A randomized controlled trial. Rheumatology64: 667–674.
44.
RosendahlA-HSchönbornKKriegT (2022) Pathophysiology of systemic sclerosis (scleroderma). Kaohsiung Journal of Medical Sciences38(3): 187–195.
45.
RossLNikpourMD’AoustJ, et al. (2023) Patient and physician global assessments of disease status in systemic sclerosis. Arthritis Care & Research75(7): 1443–1451.
46.
SamelaTRaimondiGPintoriG, et al. (2024) Clinically mild hidradenitis suppurativa: mild for whom?The British Journal of Dermatology191(6): 1026–1027.
47.
SantiagoTFernandesRFerreiraR, et al. (2024) Lessons learnt from the recent recommendations for the non-pharmacological management of systemic sclerosis. ARP Rheumatology3(Apr–Jun): 81–82.
48.
SeikowskiKWeberBHausteinUF (1995) Effect of hypnosis and autogenic training on acral circulation and coping with the illness in patients with progressive scleroderma. Hautarzt46(2): 94–101.
49.
StullCMWeaverLAValdes-RodriguezR, et al. (2018) Characteristics of chronic itch in systemic sclerosis: A cross-sectional survey. Acta Dermato-Venereologica98(8): 793–794.
50.
SurwitRSAllenLM3rdGilgorRS, et al. (1982) The combined effect of prazosin and autogenic training on cold reactivity in Raynaud’s phenomenon. Biofeedback and Self-regulation7(4): 537–544.
51.
ThombsBDKwakkenbosL, et al. (2024) Psychosocial issues and care for patients with systemic sclerosis. In: AllanoreYVargaJDentonCP (eds) Scleroderma: From Pathogenesis to Comprehensive Management. Cham: Springer International Publishing, pp.691–699.
52.
Van BeugenSSchutCKupferJ, et al. (2023) Perceived stigmatization among dermatological outpatients compared with controls: An observational multicentre study in 17 European countries. Acta Dermato-Venereologica103: adv6485.
Supplementary Material
Please find the following supplemental material available below.
For Open Access articles published under a Creative Commons License, all supplemental material carries the same license as the article it is associated with.
For non-Open Access articles published, all supplemental material carries a non-exclusive license, and permission requests for re-use of supplemental material or any part of supplemental material shall be sent directly to the copyright owner as specified in the copyright notice associated with the article.
