Research Article

Quality of Life in Patients with Morphea: A Cross-Sectional Study and a Review of the Current Literature

Table 3

Summary of previously published papers focused on QoL in morphea.

AuthorNumber of patientsAssessment of disease severityQoL scalesConclusions

Kroft et al., 2008 [11] ( years), no control group9-item skin status scale from ISDL VAS (itch, pain, and fatigue)DLQI(i) Based on the DLQI, morphea had a small impact on patient’s QoL (mean DLQI was points).
(ii) Higher fatigue, itch, and pain severity were significantly connected with a lower disease-related QoL, although both pain and itch were observed in less than 1/3 of patients and were of mild intensity.
(iii) The major limitation is the lack of control group.

Orzechowski et al., 2009 [12] ( and <18 years) vs. healthy children () and children with atopic dermatitis ()Disease severity was not assessedKINDL
CDLQI
(i) General QoL of patients with juvenile morphea was not different from healthy controls.
(ii) There were no differences in the median KINDL total or subscale scores between juvenile morphea and atopic dermatitis groups and juvenile morphea and healthy control groups.
(iii) The major limitation is the lack of disease severity assessment.

Kroft et al., 2009 [13] ( years), no control group9-item skin status scale from ISDL VAS (itch, pain, and fatigue)10-item anxiety subscale and the 6-item negative/depressed mood subscale of the ISDL(i) 38% of patients showed levels of anxiety or depressed mood comparable to those of psychiatric outpatients.
(ii) A strong correlation between higher levels of psychological distress (depressed mood and anxiety) and a more severe skin disease was observed.
(iii) The major limitations are the absence of a control group and the fact that the disease severity was assessed by patients and not by the qualified physician.

Arkachaisri et al., 2009 [14] (first phase of the study)
patients (third phase), no control group
LoSSICDLQI(i) The median CDLQI was 3.0 (IQR 2.0–3.0), and the mean was
(ii) CDLQI correlated poorly with the mLoSSI.
(iii) The major limitation is the absence of a control group and relatively small group of patients.

Saxton-Daniels and Jacobe, 2010 [15], no control groupData not presented in the paperDLQI(i) The mean DLQI was 3.5 (range 0-12), showing only a small impact of the disease on the QoL of the patients with morphea.
(ii) Adults with pediatric-onset morphea had lower QoL than other patients, especially if they also demonstrated functional impairment () and/or a large number of lesions.
(iii) The major limitation of this study was the absence of a control group and relatively small sample group.

Baildam et al., 2011 [16] (aged between 4 and 16 years with diagnosis of either localized or systemic scleroderma), no control groupModified PRES formCHAQ (physical function and pain)
CDLQI
CQOL
CHQ-PF50 (parent assessment and social function)
(i) Median CHAQ and VAS pain scores were higher among children with systemic scleroderma.
(ii) The median CDLQI was 5 (range 0-10) for the total sample, reflecting only a small impact of the disease on patients’ QoL in juvenile morphea and SSc.
(iii) The patients with juvenile morphea had higher median DLQI scores than the SSc group (5 vs. 3, respectively), although the difference was not statistically significant.
(iv) Only a moderate impairment of QoL and physical function was observed.
(v) The major limitation is the absence of any control group.

Szramka-Pawlak et al., 2013 [17]47 morphea patients and 47 healthy individualsLoSSILOT-R(i) The study was well designed and conducted.
(ii) The current and past levels of QoL in morphea patients and healthy controls were similar.
(iii) No correlation was observed between morphea severity and the QoL.

Das et al., 2014 [18] (202 adult patients and 75 children) based on the Morphea in Adults and Children (MAC) cohort, no control groupLoSCATDLQI
CDLQI
(i) Mean DLQIs among adults were for generalized morphea, for linear, and for plaque-type morphea; however, no significant association between the morphea subtype and DLQI was observed.
(ii) Mean CDLQIs among children were for generalized, for linear, and for plaque-type morphea.
(iii) There was no association between the morphea subtype and CDLQI.
(iv) Children with plaque-type morphea had a lower score in the CLDQI, but this group was not representative due to the small number of patients ().
(v) On average, children had a higher impact on QoL compared with adults.
(vi) The major limitation is the absence of a control group.

Szramka-Pawlak et al., 2014 [19], no control groupLoSSISkindex questionnaire
LOT-R (optimism level)
29-item Mini-MAC
(i) There was no association between the subtype of morphea and QoL.
(ii) The major limitation is the absence of a control group.

Condie et al., 2014 [20] (68 adults with pediatric-onset morphea and 234 patients with adult-onset morphea (based on MAC) cohort), no control groupLoSCATSF-36
DLQI
Skindex-29
(i) SF-36 scores: pediatric-onset patients had higher SF-36 scores for physical functioning ( vs. ; ), physical role ( vs. ; ), vitality ( vs. ; ), and physical component summary ( vs. ; ).
(ii) The mean DLQI score for pediatric-onset was 4.5 points and for adult-onset was 6.3 points.
(iii) Patients with pediatric-onset disease had more favorable scores as measured by Skindex-29 ( vs. ; ).
(iv) Patients with pediatric-onset disease had more favorable quality of life scores for all measures that reached statistical significance.
(v) The major limitation is the absence of a control group.

Klimas et al., 2015 [21]mRSS
LoSCAT
DLQI
SF-36
SCQ
VAS (itch, pain)
(i) DLQI data from the same cohort was presented in Das et al., 2014 [18] (see above).
(ii) Morphea had a negative impact on QoL that was similar to disorders such as eczema and rheumatoid arthritis, especially in the domain of emotions and mental health.
(iii) Lesional pain was associated with higher impairment in the SF-36 physical component summary and DLQI.
(iv) The major limitation is the predominance of plaque-type morphea over other subtypes.

Mertens et al., 2017 [22] (only patients with eosinophilic fasciitis), no control groupmRSS
LoSCAT
PhysGA-A
PhysGA-D
DLQI
SF-36
(i) The median DLQI was 3 (range 0-18), showing only a mild impact of the disease on the QoL of the patients with eosinophilic fasciitis.
(ii) The physical functioning domain of the SF-36 and DLQI scores moderately correlated with the PhysGA-D, mRSS, and LoSCAT scores.
(iii) The major limitation is the absence of a control group.

Ardalan et al., 2017 [23] (aged between 4 and 16 years), no control groupLoSCATCDLQI (dichotomization of the CDLQI)(i) The mean CLDQI score was not provided.
(ii) The median CDLQI was 1 (range: 0-17).
(iii) Each additional extracutaneous manifestation increased the likelihood of QoL impact by 37%.
(iv) Each month after the initial visit yielded 5% lower odds of QoL impact.
(v) Cutaneous activity and damage did not consistently reach statistical significance.
(vi) The major limitation is the absence of a control group.

Bali et al., 2018 [24], no control groupLoSCATDLQI(i) Median DLQI scores for generalized localized morphea was , for plaque-type morphea , and for deep morphea points.
(ii) QoL scores indicated a mild to moderate negative impact of morphea on QoL.
(iii) The major limitation is the absence of a control group.

Kunzler et al., 2019 [25] (based on MAC cohort) comparison of linear subtype with other subtypes of morphea, no control groupLoSCATDLQI
CDLQI
(i) The median DLQI for linear morphea (3, IQR 1.5-7) was similar to that for the generalized subtype (4, IQR 1-9).
(ii) The median linear CDLQI for linear morphea was 3 (IQR 1-6).
(iii) There was no association between lesion location in cosmetically sensitive sites and life quality in the linear morphea group.
(iv) Presence of functional impairment in adult patients showed a trend toward greater quality of life impact (median DLQI (IQR) with vs. without, 5 (3-8) vs. 3 (1-7); ).
(v) The major limitation is the absence of a control group.

ISDL: Impact of Chronic Skin Disease on Daily Life; VAS: Visual Analogue Scale; DLQI: Dermatology Life Quality Index; QoL: quality of life; KINDL: English language version of the German Revised Children’s Quality of Life Survey; PRES: Pediatric Rheumatology European Society; CHAQ: the Childhood Health Assessment Questionnaire; CQOL: Child Health-related Quality of Life; CHQ-PF50: Child Health Questionnaire; SSc: systemic scleroderma; LoSSI: Localized Scleroderma Severity Index; SF-36: Short Form-36; LOT-R: Cantril’s Ladder Life Orientation Test-Revised; LoSCAT: Localized Scleroderma Cutaneous Assessment Tool; MAC: the Morphea in Adults and Children; Mini-MAC: Mental Adjustment to Cancer Scale; mRSS: modified Rodnan Skin Score; SCQ: Self-Administered Comorbidity Questionnaire; PhysGA-A: the Physician Global Assessment of Disease Activity; PhysGA-D: Physician Global Assessment of Disease Damage.