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, healthrelated 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. JEADV 2017, 31, 30-37 32 Lis-Swie z ty et al. JEADV 2017, 31, 30-37 34 Lis-Swie z ty et al. JEADV 2017, 31, 30-37 36Lis-Swie z ty et al.

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.

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.

BackgroundLocalized 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.MethodsBefore 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.ResultsThe 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.ConclusionsDespite 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 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.