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Interferon-alpha seems to be the best initial treatment for ECD. Since 2012, more than 200 patients worldwide with multi-system or refractory ECD have benefitted from highly effective therapy with BRAF and MEK inhibitors. Targeted therapies have an overall, robust and reproducible efficacy in ECD, with no acquired resistance to date, but their use may be best reserved for the most severe manifestations of the disease, as they may be associated with serious side-effects and as yet unknown long-term consequences. Copyright © 2020 American

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The methodological quality of the studies was "fair" for most of the psychometric characteristics analyzed (43 items), with 24 properties scored as "poor," 5 as "good," and 3 as "excellent." Conclusions Different instrument characteristics-such as the scope and population with which the instrument will be used, its dimensions, the number of items, and the evidence shown in the evaluation of each psychometric property-should be considered by clinicians and researchers to decide which instrument is the best to measure the construct of patie