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Objective: The 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., Methods: We 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., Results: Thirteen 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., Conclusion: Overall, 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., (© 2023 The Authors. Arthritis Care & Research published by Wiley Periodicals LLC on behalf of American College of Rheumatology.)

Purpose: Anchoring vignettes (AVs) are a promising measurement technique to reduce bias in patient-reported outcome (PRO) measures by helping researchers understand differences in how individuals and groups interpret response options. However, little attention has been paid to ensure quality development of AVs, and their performance has not been well assessed in pediatric populations. In this study, we explore the application of a rigorous development process for AVs based upon current standards for PROs, as well as feasibility of AVs when administered to children and adolescents., Methods: We developed AVs using a rigorous, patient-centered mixed methods process including three phases: (1) development, (2) a pilot study, and (3) a field test. Our proposed process included the generation of a conceptual framework based on the PRO, the Localized Scleroderma Quality of Life Instrument, and numerous vignette-specific considerations. We qualitatively explored readability and comprehension of the AVs (pilot study) and then analyzed ranking patterns within vignette sets (field test)., Results: Four sets of four vignettes were developed. Revisions were suggested at each phase of development. The pilot study demonstrated that children ≥ 10 years had no trouble indicating understanding of the AVs. In the field test, although appropriate rankings of vignettes were generally demonstrated by participants, the percentage of tied rankings was higher than expected in this pediatric group., Conclusions: This work supports the need for rigorous developmental standards for AVs, as each stage of development suggested revisions. Additionally, AVs showed initial promise for use with pediatric populations; general feasibility and understanding were supported.

Background: Localized Scleroderma (LoS) is an autoimmune connective tissue disease that affects skin and less commonly subcutaneous tissues. The illness occurs in children and adults, and may have a serious impact on health-related quality of life (HRQoL). The goal of this study was to explore what factors might deteriorate scores on HRQoL measures in adult LoS patients., Methods: Detailed information on the demographic and clinical features of LoS patients was collected. The HRQoL was assessed using Skindex-29 and Short form 36 (SF-36) questionnaires., Results: Thirty three women and seven men with LoS were enrolled. Female gender negatively influenced scores for the emotion subscale of Skindex-29. Multiple-factor linear regression analysis confirmed, as with single-factor analysis, that the causes of low SF-36 physical component score (PCS) were subjective symptoms (pruritus, pain, paresthesia), musculoskeletal manifestations and older age at the time of survey, while rural area of residence negatively affected the SF-36 mental component score. Additionally, single-factor analysis showed that the SF-36 PCS was related to the LoS cutaneous assessment tool (LoSCAT) summary score., Conclusions: Apart from a clinical presentations, sociodemographic characteristics, including older age, female gender and living conditions, may impair HRQoL of LoS patients. Further studies that will examine the role of these factors for physical and mental functioning of adults with LoS are needed.

Objective: The aim of the study was to evaluate QoL in patients suffering from morphea. Material and Methods . Sixty-five patients with morphea were recruited into this cross-sectional, prospective parallel study. QoL among adult patients was assessed with the Dermatology Life Quality Index (DLQI) and Euro-QoL-5D questionnaire; patients aged <17 years used the Children's Dermatology Life Quality Index (CDLQI). The severity of morphea was assessed using the Localized Scleroderma Cutaneous Assessment Tool. The results of QoL and its association with disease severity were compared between patients with various morphea subtypes., Results: The mean DLQI scoring was 3.8 ± 4.1 points and the CDLQI was 2.3 ± 3.0. The mean value of Visual Analogue Scale thermometer (EQ VAS) was 66.9 ± 17.5 points. The disease activity of morphea based on mLoSSI correlated significantly with QoL impairment according to the DLQI ( R = 0.41, p = 0.001). No significant correlation was observed between morphea-induced damage and QoL ( p = 0.99)., Conclusions: Evaluation of QoL in patients with morphea is still challenging due to lack of good assessment tools dedicated specifically for morphea patients. In general, QoL in morphea patients is significantly correlated with the disease activity, but not with disease-induced skin damage., Competing Interests: The authors declare that they have no conflicts of interest., (Copyright © 2020 Justyna Szczęch et al.)

Purpose of Review: Linear scleroderma is the most common subtype of localized scleroderma (LoS) in children. It can be associated with extracutaneous manifestations and long-term sequelae. Thus, appropriate diagnosis and management are key to improve the prognosis. In this review, we summarize the most relevant recent publications for the diagnosis, evaluation of disease activity and adequate management of patients with linear scleroderma., Recent Findings: There are specific clinical features that indicate activity in LoS; dermoscopy and Wood's lamp may be useful. Summarizing, scoring methods seem to provide the most adequate assessment of LoS; but several biomarkers that correlate with activity have been studied: E-selectin and IL-2 receptor, CD34+ dermal dendritic cells and Th/Th1 immunophenotype with decreased T helper (Th2), T regulatory (Tregs), B and natural killer (NK) cells. Recent studies propose hydroxychloroquine monotherapy and tocilizumab as potential therapeutic options., Summary: Clinical evaluation, both physical exam and history, is the most important aspect in diagnosing and assessing activity of linear scleroderma. Clinical scoring methods may be most useful for evaluation of activity; eventually, other biomarkers could be relevant in clinical practice. For most patients with linear scleroderma, the first choice of treatment is methotrexate, but physical therapy, plastic surgery and/or orthopedic management are key to improve residual limitations and quality of life. VIDEO ABSTRACT: http://links.lww.com/MOP/A35.

Bullous morphea is rare clinical variant of localized scleroderma characterized by the formation of bullae on sclerotic morphea plaques. Severe disease may be highly disabling and greatly impair quality of life. Current treatment strategies are based on anecdotal reports of clinical experience and include topical corticosteroids, methotrexate and phototherapy. Herein, we describe the case of a 56-year-old woman with progressive bullous sclerotic lesions who was successfully treated with mycophenolate mofetil after treatment failure with psoralen plus ultraviolet A therapy, ultraviolet A1 phototherapy, and methotrexate. Treatment with mycophenolate mofetil halted disease progression after 8 weeks. No major adverse effects were recorded in a 3-year follow-up with continuous treatment. This case suggests mycophenolate mofetil may be considered as an alternative for the treatment of resistant bullous morphea lesions. J Drugs Dermatol. 2018;17(10):1123-1125.

Background: Morphea can lead to considerable cosmetic or functional impairment; nevertheless, health-related quality of life (HRQoL) is rarely documented in adult morphea patients., Objective: To investigate the impact of morphea on HRQoL and to identify determinants of impaired HRQoL., Methods: A cross-sectional study has been carried out among adult morphea patients. HRQoL was evaluated by the Dermatology Life Quality Index (DLQI). The modified Localised Scleroderma Skin Severity Index (mLoSSI) and the Localised Scleroderma Damage Index (LoSDI) were applied to evaluate disease activity and damage, respectively. Physician Global Assessment of Activity and Damage (PGA-A, PGA-D) were also completed. Determinants of HRQoL were analysed by multiple regression., Results: A total of 101 patients (84% females) entered the study, with a mean age of 56.8 ± 14.8 years. Median mLoSSI, LoSDI, PGA-A and PGA-D scores were 8, 5, 9 and 9 points, respectively. Patients with generalised localised (51%) and plaque-type morphea (45%) had median total DLQI scores of 4 and 1, respectively. Embarrassment (53%), itchy or painful skin (46%), and clothing issues (43%) were the most commonly reported problems in the DLQI. Female gender, generalised morphea, higher disease activity (PGA-A score) and involvement of hands and/or feet were significant predictors of impaired HRQoL (p < 0.05)., Conclusion: This study represents the largest sample of adult morphea patients surveyed about their HRQoL in Europe. The frequent occurrence of embarrassment warrants an increased attention to improve patients' mental health. Care must be taken in case of involvement of functionally sensitive areas, as these cases might require more intensive treatment.

Background: Facial morphoea is a chronic inflammatory skin disorder, typically presenting in childhood and adolescence, which can be disfiguring, and which has been suggested to cause mild-to-moderate impairment in quality of life., Objectives: To explore the everyday experiences of children with facial morphoea by examining the psychosocial impact of living with facial morphoea and how children and their families manage its impact., Methods: We used a qualitative, social constructionist approach involving focus groups, in-depth interviews and drawing activities with 10 children with facial morphoea aged 8-17 years and 13 parents. Interpretive thematic analysis was utilized to examine the data., Results: Children and parents reported on the stress of living with facial morphoea, which was related to the lack of knowledge about facial morphoea and the extent to which they perceived themselves as different from others. Self-perceptions were based on the visibility of the lesion, different phases of life transitions and the reactions of others (e.g. intrusive questioning and bullying). Medication routines, and side-effects such as weight gain, added to the stress experienced by the participants. To manage the impact of facial morphoea, children and their parents used strategies to normalize the experience by hiding physical signs of the illness, constructing explanations about what 'it' is, and by connecting with their peers., Conclusions: Understanding what it is like to live with facial morphoea from the perspectives of children and parents is important for devising ways to help children with the disorder achieve a better quality of life. Healthcare providers can help families access resources to manage anxiety, deal with bullying and construct adequate explanations of facial morphoea, in addition to providing opportunities for peer support., (© 2018 British Association of Dermatologists.)

The purpose of this study was to describe the experiences of fathers with scleroderma. Ten fathers with scleroderma were interviewed by telephone. Interviews were tape-recorded and transcribed verbatim. Two key themes emerged related to the emotional impact of the illness and the day to day realities of the illness with the unpredictability and rareness of the illness leading to ongoing feelings of isolation and fear of mortality. The negative influences of being a father with scleroderma included the inability of the fathers to participate in physical activities with their children such as outdoor sports and throwing balls. Being able to spend quality time with the child was a positive influence of the illness.

Objective: Localized scleroderma can negatively affect children's quality of life (QoL), but predictors of impact have not been well described. We sought to identify predictors of QoL impact in juvenile localized scleroderma patients., Methods: We analyzed longitudinal data from a single-center cohort of juvenile localized scleroderma patients, using hierarchical generalized linear modeling (HGLM) to identify predictors of QoL impact. HGLM is useful for nested data and allows for evaluation of both time-variant and time-invariant predictors., Results: The number of extracutaneous manifestations (ECMs; e.g., joint contracture and hemifacial atrophy) and female sex predicted negative QoL impact, defined as a Children's Dermatology Life Quality Index score >1 (P = 0.019 for ECMs and P = 0.002 for female sex). As the time since the initial visit increased, the odds of reporting a negative QoL impact decreased (P < 0.001)., Conclusion: Our results suggest that ECMs, sex, and time since initial visit are more predictive of QoL impact in localized scleroderma than cutaneous features. Further study is required to determine which ECMs have the most impact on QoL, which factors underlie sex differences in QoL in localized scleroderma, and why increasing the time since the initial visit appears to be protective. An improved understanding of predictors of QoL impact may allow for the identification of patients at risk of poorer outcomes and for the tailoring of treatment and psychosocial support., (© 2016, American College of Rheumatology.)

Background/objectives: Cosmetic camouflage is known to improve quality of life in adults. Few data are available regarding cosmetic camouflage in children, and thus it is not often selected as a mode of treatment. We sought to determine whether cosmetic camouflage leads to improved quality of life of pediatric patients with visible dermatoses and their parent or primary caregiver., Methods: Patients aged 5 to 17 years with visible skin disease and their parent were assessed with the Children's Dermatology Life Quality Index (CDLQI) and the Family Dermatology Life Quality Index (FDLQI) before and after consultation regarding cosmetic camouflage., Results: Twenty-two children with skin conditions were included in the study. The mean CDLQI decreased from 6.82 (SD = 1.28) to 3.05 (SD = 0.65; P = .0014), while the mean FDLQI decreased from 7.68 (SD = 1.15) to 4.68 (SD = 0.92; P = .0012)., Conclusions: Our study highlighted improvement in quality of life in patients with skin disorders who were managed with cosmetic camouflage., (© The Author(s) 2015.)

This systematic review was conducted to evaluate the present state of body image research in children with morphea and the extent of body image distress in this population. Only five studies met inclusion criteria. Disease-related skin changes in children with morphea typically were associated with, at most, mild levels of body image distress., (© 2015 Wiley Periodicals, Inc.)

Background: Little is known about the health-related quality of life (HRQoL) of patients with morphoea, and previous studies have yielded conflicting results., Objectives: To determine the impact of morphoea on HRQoL, and clinical and demographic correlates of HRQoL in adults., Methods: This was a cross-sectional survey (n = 73) of the Morphea in Adults and Children cohort., Results: Morphoea impairs HRQoL in adults. Patients were most impaired by emotional well-being and concerns that the disease would progress to internal organs. Patients with morphoea had worse skin-specific HRQoL than those with nonmelanoma skin cancer, vitiligo and alopecia (lowest P < 0·01). Participants had significantly worse global HRQoL scores than the general U.S. population for all subscales (all P < 0·01), with the exception of bodily pain. Comorbidity (r = 0·35-0·51, all P < 0·01), and symptoms of pruritus (r = 0·38-0·64, all P < 0·01) and pain (r = 0·46-0·74, all P < 0·01) were associated with impairment in multiple domains of skin-specific and global HRQoL. Physician-based measures of disease severity correlated with patient-reported HRQoL., Conclusions: Patients with morphoea experience a negative impact on HRQoL, particularly if symptoms (pruritus and pain) or concerns regarding internal manifestations are present. Providers should be aware of this when evaluating and treating patients., (© 2014 British Association of Dermatologists.)

Background: Lack of agreement on how to accurately capture disease outcomes in localized scleroderma (LS) has hindered the development of efficacious treatment protocols. The LS Cutaneous Assessment Tool (LoSCAT), consisting of the modified LS Skin Severity Index (mLoSSI) and the LS Damage Index, has potential for use in clinical trials., Objective: The goal of this article is to further evaluate the clinical responsiveness of the LoSCAT. Based on the modifiable nature of disease activity versus damage, we expected the mLoSSI to be responsive to change., Methods: At 2 study visits, a physician completed the LoSCAT and Physician Global Assessment (PGA) of Disease Activity and of Disease Damage for 29 patients with LS. Spearman correlations were used to examine the relationships between the change in the LoSCAT and the PGA scores. To evaluate contrasted group validity, patients were grouped according to disease activity classification and change scores of groups were compared. Minimal clinically important differences were calculated and compared with the standard error of measurement., Results: Change in the mLoSSI score correlated strongly with change in the PGA of Disease Activity score, whereas change in the LS Damage Index score correlated weakly with change in the PGA of Disease Damage score. The mLoSSI and PGA of Disease Activity exhibited contrasted group validity. Minimal clinically important differences for the activity measures were greater than the respective standard errors of measurement., Limitations: Only 2 study visits were included in analysis., Conclusion: This study gives further evidence that the LoSCAT, specifically the mLoSSI, is a responsive, valid measure of activity in LS and should be used in future treatment studies., (Copyright © 2013 American Academy of Dermatology, Inc. Published by Mosby, Inc. All rights reserved.)

The clinical course of localized scleroderma may consist of bodily deformations, and bodily functions may also be affected. Additionally, the secondary lesions, such as discoloration, contractures, and atrophy, are unlikely to regress. The aforementioned symptoms and functional disturbances may decrease one's quality of life (QoL). Although much has been mentioned in the medical literature regarding QoL in persons suffering from dermatologic diseases, no data specifically describing patients with localized scleroderma exist. The aim of the study was to explore QoL in localized scleroderma patients and to examine their coping strategies in regard to optimism and QoL. The study included 41 patients with localized scleroderma. QoL was evaluated using the SKINDEX questionnaire, and levels of dispositional optimism were assessed using the Life Orientation Test-Revised. In addition, individual coping strategy was determined using the Mini-MAC scale and physical condition was assessed using the Localized Scleroderma Severity Index. The mean QoL score amounted to 51.10 points, with mean scores for individual components as follows: symptoms = 13.49 points, emotions = 21.29 points, and functioning = 16.32 points. A relationship was detected between QoL and the level of dispositional optimism as well as with coping strategies known as anxious preoccupation and helplessness-hopelessness. Higher levels of optimism predicted a higher general QoL. In turn, greater intensity of anxious preoccupied and helpless-hopeless behaviors predicted a lower QoL. Based on these results, it may be stated that localized scleroderma patients have a relatively high QoL, which is accompanied by optimism as well as a lower frequency of behaviors typical of emotion-focused coping strategies.

Objective: there have been few studies of quality of life in childhood scleroderma and these focused predominantly on self-perception and the influence of skin lesions. Our cross-sectional study aimed to describe the influence of childhood scleroderma on physical function and quality of life in relation to clinical and demographic measures., Methods: children with either localized scleroderma or systemic sclerosis (SSc) attending pediatric rheumatology clinics, together with their parents or guardians, were asked to complete a set of 4 validated measures. Clinical and demographic data were provided by consultant pediatric rheumatologists., Results: in total, 28 children and their parents/guardians participated in the study (68% female, median age 13 yrs; 86% localized scleroderma, 14% SSc). The median Child Health Assessment Questionnaire (CHAQ) score was 0.1 (range 0-3, 0 indicating no impairment), the median Child Dermatology Life Quality Index (CDLQI) score was 5 (range 0-30, 0 indicating no impairment), and the median Child Quality of Life Questionnaire (CQOL) function score was 26 (range 0-105, 0 indicating no impairment). Family activity, measured by the Child Health Questionnaire (CHQ-PF50), was also moderately impaired by scleroderma, with a median score of 83 (0-100, 100 indicating no impairment)., Conclusion: scleroderma had only a moderate effect on quality of life and physical function as measured by the 4 validated instruments. Although a small number of children reported greater impairment, this is an encouraging finding, given its potential disfiguring and debilitating effects.

This study examines the utility of the aesthetic and reconstructive categorization for making treatment decisions in patients seeking facial surgery. A total of 360 patients with aesthetic or combined functional aesthetic deficits were included. Validated psychological scales were used as outcome measures. All subjects showed clinically significant levels of appearance-related distress, with highest levels in the aesthetic and lowest levels in the functionally impaired group. Significant gender differences were not found on any psychological measures. A small negative correlation was found between age and distress. These findings challenge the validity of restricting treatment on the basis of an aesthetic distinction, since this is the group demonstrating the highest level of need. Neither age nor gender is a reliable discriminator. Subjective assessment of noticeability of disfigurement and associated psychological distress may be more useful in prioritizing treatment in systems with limited resources.

Objective: To examine the level of psychological distress and factors contributing to distress in patients with morphea or eosinophilic fasciitis., Design: Cross-sectional study., Setting: Dermatology outpatient clinic of a university hospital., Participants: Of 120 patients with morphea or eosinophilic fasciitis diagnosed between December 1, 1994, and July 15, 2007, who were enrolled in the study, only 74 completed questionnaires were suitable for data analysis., Main Outcome Measures: Self-reported responses on the Impact of Chronic Skin Diseases on Daily Life scale measure psychological distress, specifically anxiety and depressed mood., Results: Psychological functioning was generally impaired in patients with skin disease, particularly among patients with generalized morphea and eosinophilic fasciitis. Twenty-eight patients (38%) were at risk of depression or anxiety. Higher levels of psychological distress were significantly related to greater severity of skin disease; more pain and fatigue; impact of disease on daily life; more perceived stigmatization; illness cognitions of greater helplessness; and less acceptance and less perceived social support., Conclusions: Physical and psychosocial aspects play a substantial role in the quality of life for patients with morphea. Physicians should be encouraged to assess the physical and psychosocial factors when treating patients with sclerotic skin diseases. This approach could improve quality of life and ultimately lead to improved dermatological treatment outcomes.

Objectives: To examine the health-related quality of life (HRQOL) of children with juvenile localized scleroderma (JLS) and to compare them with patients with atopic dermatitis (AD) and healthy controls., Methods: The cohorts were identified through a diagnostic index and were seen between January 1996 and December 2006. We identified 81 JLS patients to whom we age- and sex-matched 75 AD patients and 75 healthy controls. All patients were mailed a survey containing the English-language version of the German Revised Children's Quality of Life Questionnaire (KINDL) and the Children's Dermatology Life Quality Index (CDLQI). Linear regression models, adjusted for age and sex, examined differences in the KINDL and CDLQI scores., Results: Survey completion rates in the JLS, AD and healthy control groups were 40, 28 and 44%, respectively. There was no difference in KINDL scores between JLS vs AD (73 vs 74, P = 0.3) and JLS vs healthy controls (73 vs 74, P = 0.8). However, CDLQI scores showed some impairment in HRQOL in JLS patients as compared with a healthy reference population, but not to the degree seen in AD (2 vs 4, P = 0.05). An exploratory analysis showed that HRQOL did not differ among the types of JLS with either measure., Conclusion: JLS patients have some impairment in skin disease-specific HRQOL when compared with a healthy reference population, but not as severe as that seen in AD patients. Overall HRQOL in this JLS cohort was as good as healthy controls, a reassuring finding for patients, families and healthcare providers.

Background: The LPG technique, also known as Endermology treatment, is a noninvasive technique consisting of a tissue mobilization process in which a skin fold is created between two rollers, stretching the underlying tissue and mobilizing the fold. The LPG technique is very effective in treating scars. Because the lesions of morphea or circumscribed scleroderma are similar to atrophic scars, it seemed reasonable to treat them with a method proven helpful for scars., Materials and Methods: We treated 17 lesions of 10 patients (four males and six females) with the diagnosis of morphea ranging in age from 17 to 78 years (mean age 55 years) and investigated and documented the evolution of their lesions and changes in their quality of life., Results: In all patients there was a large improvement in the clinical appearance of the lesions, the induration and the pain. Elasticity was particularly increased, not only based on clinical findings but also as documented with objective assessment. The acceptability of the treatment was good and the patients reported an improved quality of life., Conclusion: The LPG technique (Endermology) is an adjunctive treatment for morphea. It cannot eliminate the disease but can relieve the pain, soften the skin and improve the quality of life for these patients., (Copyright 2004 European Academy of Dermatology and Venereology)

Objective: To evaluate health-related quality of life (HRQOL) in patients with systemic sclerosis (SSc) using the Short Form 36 (SF-36) and to correlate SF-36 scores with clinical and biologic markers., Methods: The SF-36 was administered to 24 controls and 24 SSc patients. SSc patients also were evaluated for subset (limited SSc [lSSc] and diffuse SSc [dSSc]), age, disease duration, angiotensin-converting enzyme (ACE) levels, autoantibodies, and skin and internal organ involvement., Results: The physical summary score (PSS) was lower in SSc patients than in controls (P < 0.05), whereas the mental summary score (MSS) was higher in dSSc than in lSSc patients (P < 0.05). Five of 8 single SF-36 domain scores were lower in SSc patients than in controls (P < 0.05). Vitality was higher in dSSc than in controls (P < 0.001). In SSc, elder age correlated with lower PSS; low ACE levels and high skin score correlated with higher general mental health and role limitations due to physical problems, respectively (P < 0.05). Patients with heart involvement had higher scores in general health perceptions (P < 0.05)., Conclusion: The SF-36 shows that HRQOL is impaired in patients with SSc. Higher scores in MSS and vitality in patients with dSSc and correlations of high SF-36 scores with specific organ involvement suggest that SSc patients with severe disease are more able to cope with HRQOL modification.

Background: Little is known about the experience of living with a rare disease and how people with rare diseases cope with not only the disease but also the reactions of others. Scleroderma is a rare chronic connective tissue disease that results in fibrotic changes involving all or some organs of the body. The two types of scleroderma are systemic scleroderma, which involves the skin and internal organs and is the more serious type, and local scleroderma, which attacks the skin and surrounding tissues. Some people with scleroderma have signs that are visible to outsiders, while others have invisible signs. Having this chronic condition and being different from the general population may subject people with scleroderma to stigmatization by others., Aim: The aim of this study was to understand, from the individual's perspective, the experience of living with scleroderma., Method: Focus group interviews were conducted with two groups of individuals with scleroderma. Because of the rarity of the disease and the illness of the participants, only two groups were held. The same questions were asked of both groups. A moderator and assistant guided the groups., Findings: Data analysis revealed five themes: physical manifestations, disclosure/non-disclosure to others, living, being normal and facing the future. The data are discussed in light of participants' having visible signs, invisible signs and the rarity of their condition. For those with visible signs, disclosure was automatic. They were conscious of being different from others without scleroderma. Those with invisible signs managed their disease information in such a way as to minimize the stigma of being different. The rarity of the disease added the problem of others not understanding their difficulties. Those who disclosed their disease not only had to deal with the reactions of others, but faced the additional burden of having to explain their condition., Conclusion: Nurses may have little knowledge about scleroderma. It is possible that they, through their ignorance of such rare conditions, may stigmatize individuals. Through understanding about rare diseases will they be able to teach patients the skills necessary to help them cope with their symptoms, as well as the reactions of others to their diagnosis and appearance.

Body image dissatisfaction and its relationship to psychosocial function were investigated in 127 women with scleroderma Results indicated elevated body image dissatisfaction, with participants reporting higher levels than a sample of patients with severe burn injuries. Age, skin tightening above the elbows, and functional disability were related to heightened body image dissatisfaction, suggesting that younger patients with more severe disease may be at greatest risk for developing body image concerns. Path analysis revealed that depression mediated the relationship between body image dissatisfaction and psychosocial function. Results suggest that body image dissatisfaction is a significant concern in women with scleroderma and should be assessed routinely. Early identification and treatment of body image dissatisfaction may help prevent the development of depression and psychosocial impairment in this population.

Objective: To document disease activity and functional status in patients with scleroderma (systemic sclerosis [SSc]) and Raynaud's phenomenon (RP) and to determine the sensitivity to change, reliability, ease of use, and validity of various outcome measures in these patients., Methods: Patients with SSc and moderate-to-severe RP participating in a multicenter RP treatment trial completed daily diaries documenting the frequency and duration of RP attacks and recorded a daily Raynaud's Condition Score (RCS). Mean scores for the 2-week periods prior to baseline (week 0), end of trial (week 6), and posttrial followup (week 12) were calculated. At weeks 0, 6, and 12, physicians completed 3 global assessment scales and performed clinical assessments of digital ulcers and infarcts; patients completed the Health Assessment Questionnaire (HAQ), the Arthritis Impact Measurement Scales 2 (AIMS2) mood and tension subscales, 5 specific SSc/RP-related visual analog scales (VAS), and 3 other VAS global assessments. We used these measures to document baseline disease activity and to assess their construct validity, sensitivity to change, and reliability in trial data., Results: Two hundred eighty-one patients (248 women, 33 men; mean age 50.4 years [range 18-82 years]) from 14 centers participated. Forty-eight percent had limited cutaneous SSc; 52% had diffuse cutaneous SSc. Fifty-nine patients (21%) had digital ulcers at baseline. Patients had 3.89 +/- 2.33 (mean +/- SD) daily RP attacks (range 0.8-14.6), with a duration of 82.1 +/- 91.6 minutes/attack. RCS for RP activity (possible range 0-10) was 4.30 +/- 1.92. HAQ scores (0-3 scale) indicated substantial disability at baseline (total disability 0.86, pain 1.19), especially among the subscales pertaining to hand function (grip, eating, dressing). AIMS2 mood and tension scores were fairly high, as were many of the VAS scores. Patients with digital ulcers had worse RCS, pain, HAQ disability (overall, grip, eating, and dressing), physician's global assessment, and tension, but no significant difference in the frequency of RP, duration of RP, patient's global assessment, or mood, compared with patients without digital ulcers. VAS scores for digital ulcers as rated by the patients were not consistent with the physician's ratings. Factor analysis of the 18 measures showed strong associations among variables in 4 distinct domains: disease activity, RP measures, digital ulcer measures, and mood/tension. Reliability of the RCS, HAQ pain and disability scales, and AIMS2 mood and tension subscales was high. The RP measures demonstrated good sensitivity to change (effect sizes 0.33-0.76)., Conclusion: Our findings demonstrate that the significant activity, disability, pain, and psychological impact of RP and digital ulcers in SSc can be measured by a small set of valid and reliable outcome measures. These outcome measures provide information beyond the quantitative metrics of RP attacks. We propose a core set of measures for use in clinical trials of RP in SSc patients that includes the RCS, patient and physician VAS ratings of RP activity, a digital ulcer/infarct measure, measures of disability and pain (HAQ), and measures of psychological function (AIMS2).

We evaluated the self-esteem and quality of life of 47 children with morphea with the use of the Harter self-perception profile for children and Visual Analog Scale. Most children with morphea have normal self-worth and a high quality of life. Morphea, like some other childhood chronic illnesses, does not impair self-esteem.

Although quite a rare pediatric disease, linear scleroderma (LS), a chronic disease, may leave scarring and may have devastating effects on a child and family. This article provides an overview of the disease, presents a case history of a boy with LS, and discusses how nurses and other health-team members assisted the child and family in managing the illness. The description of the nursing care is based on the Neuman systems model and on the nursing diagnoses of fear, disturbed self-concept, and impaired physical mobility.