A systematic review of tools for determining activity of localized scleroderma in paediatric and adult patients

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.