Search

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.

Background: Clinical characteristics of the lesions are used to identify activity and damage in localized scleroderma (LoS). For high-frequency ultrasound (HF-US), the features of active lesions were described., Materials and Methods: Clinical signs of activity and damage in LoS lesions were assessed with the use of Localized Scleroderma Cutaneous Assessment Tool (LoSCAT) and HF-US by two examiners independently. All US images were obtained using a 20 MHz HF-US (DermaLab System, Cortex Technology, Hadsund, Denmark). The dermal thickness (DT) and echogenicity (intensity score, IS) of the LoS lesional dermis were measured in the area of each lesion with the highest score for erythema (ER), skin thickness (ST), and dermal atrophy (DAT). Measurements were compared to the site-matched unaffected skin. The relative difference of DT and IS values was calculated between each lesion and its normal control for comparison among different clinical scores for ER, ST, and DAT., Results: A total of 92 lesions in 40 adult patients were examined with HF-US. Thirty one lesions were erythematous, 26 were in sclerosis, and 35 were in atrophy. A correlation between the clinical evaluation of the LoS lesions and US measurements was found. The sensitivity and specificity of HF-US were 97% and 90%, respectively. The positive predictive value was 83%, negative predictive value-98%. Interrater reliability was excellent for LoSCAT and HF-US findings., Conclusion: High-frequency ultrasound allows an accurate assessment of the inflammatory and fibrotic skin lesions in LoS., (© 2019 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Background: The disease phase in localized scleroderma (LoS) is commonly assessed by clinical investigation using the LoS cutaneous assessment tool (LoSCAT). There is a need for more objective methods for assessing the degree of activity/inflammation in LoS. The aim of our work was to evaluate the correlation between severity of the LoS lesions scored using LoSCAT and the degree of hyperthermia observed with infrared thermography (IRT)., Materials and Methods: The LoS lesions were examined using the LoSCAT and IRT independently by two examiners. The average temperature (Tavg) of the LoS lesional dermis were measured in the area of each lesion with the highest score for erythema (ER), skin thickness (ST), dermal atrophy (DAT) and subcutaneous atrophy (SAT). Measurements were compared to the contralateral healthy skin. The difference of the Tavg (ΔTavg) was calculated between each lesion and its normal control., Results: One hundred and four LoS lesions were examined in 40 adults. Thirty-one lesions were erythematous, 26 were sclerotic, 35 presented as atrophy of the dermis and 11 as atrophy of the subcutaneous tissue. The sensitivity and specificity of IRT for detecting activity/inflammation were 80.7% and 86.3%, respectively. The positive predictive value was 71%, negative predictive value 91%. Statistically significant positive correlation was found between the ΔTavg and the clinical ER and DAT scores., Conclusion: IRT may be a useful method for assessing erythematous LoS lesions and quantifying the degree of activity/inflammation. Knowing the patient, false positive results attributable to more severe degree of skin and subcutaneous fat atrophy can be easily recognized., (© 2018 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

Background: Localized scleroderma (LoS) affects both children and adults and is associated with permanent functional and cosmetic impairment, and reduced quality of life predominating in adults. The Localized Scleroderma Cutaneous Assessment Tool (LoSCAT) is a clinical instrument designed to measure an activity and damage of LoS. It has been validated for use with pediatric LoS patients. This study assessed the validity and reliability of the LoSCAT adapted for use in adults., Methods: Before the initiation of the study two examiners participated in an intensive training course carried out by an expert in LoS. Appendices describing each LoSCAT domain were prepared. Features determining disease activity and damage in adult LoS patients were identified to properly evaluate the physician (Phys) and patient (Pt) global assessment (GA) of disease activity (A)/severity (S) and damage (D), which were used to assess convergent validity of the LoSCAT. Correlations of physician- and patient-derivied measures with Skindex-29 were also analysed., Results: The study included 40 adult LoS patients (33 females and 7 males) with different subtypes of LoS. Intra and inter-rater reliability of the LoSCAT was found to be excellent. Positive correlations were observed between the PhysGA-A, PhysGA-D, PtGA-A and the LoSCAT's domains, while no correlations between them and the PtGA-D were found. There were no relationships between LoSCAT's components and Skindex-29., Conclusions: Despite the LoSCAT is a reliable tool for an assessment of cutaneous lesions, additional health status instruments are necessary to a holistic approach to LoS in adults.

Background: Localized scleroderma is a rare inflammatory skin disorder that affects the skin and sometimes underlying subcutaneous tissue, muscles or bones. The disease has two modes of onset: juvenile- (JLS) and adult-onset (aLoS). Clinical features have impact on diagnostic and treatment recommendations, but no consensus on the disease management depending on the age at diagnosis was given., Objective: To identify these features which differentiate aLoS from JLS., Methods: A review of the literature was carried out using the MEDLINE to identify studies assessing demographics, subtype distribution, extracutaneous manifestations, comorbidities, delay at diagnosis and outcome in JLS and aLoS. Non-English articles, reviews, case reports, treatment trials that did not investigate long-term outcomes and studies with the fused data for children and adults were excluded. The analysed papers were published between June 1986 and December 2016., Results: Fifty-five studies describing JLS or/and aLoS were included for analysis. Female: male ratio in aLoS was higher than in JLS. Adults presented with plaque and generalized subtype more often than paediatric patients. Linear subtype, musculoskeletal, neurologic and ophthalmologic involvement were more frequent, among children. aLoS was likely to be associated with increased prevalence of lichen sclerosus. There was significant delay in diagnosis in both groups of patients. Recurrences of disease were independent of its onset mode. aLoS patients had more poor quality of life scores than did JLS patients. As only a few studies or no studies assessing specified disease subtypes were identified, this limited the ability to compare the adult and paediatric patients with these subtypes., Conclusion: Despite more favourable course of aLoS, a thorough understanding of the broad spectrum of domains related to physical, mental, emotional and social functioning of patients seems to be important to the proper management of disease. LoS heterogeneity makes it important to develop universal classification criteria., (© 2017 European Academy of Dermatology and Venereology.)

Localized scleroderma (LoS) is a rare inflammatory skin disorder that affects the dermis and sometimes subcutaneous tissues. LoS can have very long periods of quiescence followed by reactivation, but the progression or activity of the disease is difficult to measure. To review the measuring tools used for the evaluation of LoS activity, to choose the most appropriate technique to facilitate progress towards properly assessing the disease, a systematic review of the literature was carried out using the PubMed MEDLINE. Sixty-three studies describing groups of children, adults or both were reviewed and included in the analysis. Case reports were excluded. The analysed papers were published between June 1986 and February 2016. Data were extracted with a focus on instruments measuring the clinical signs of LoS, health-related quality of life (HRQoL), laboratory tests and imaging techniques. Perusal of the literature confirmed that clinical characteristics of the lesions were used to identify activity and scoring systems that focused on a series of signs, and were initially validated in cases of childhood-onset disease; however, there were no data concerning the adult-onset form of the disease. Adult patients with LoS scored lower on HDLQI than those with paediatric-onset LoS. No validated biological markers were available as correlative laboratory parameters of LoS activity. For infrared thermography, ultrasound and other imaging techniques, the features of active lesions were described, but were only useful with appropriate clinical correlation. Measuring tools have not been prospectively validated yet. Summarizing, scoring methods seem to provide the most adequate assessment of LoS and deserve to be further investigated. Combined imaging techniques create optimal conditions for the proper interpretation of the temperature at the skin surface, as well as the structure and vascularity of LoS lesions. Additional scores, musculoskeletal or neuroimaging techniques and laboratory parameters are needed for the specific disease subtypes to monitor extracutaneous manifestations., (© 2016 European Academy of Dermatology and Venereology.)