AUTHOR=Papara Cristian , De Luca David A. , Bieber Katja , Vorobyev Artem , Ludwig Ralf J. 

TITLE=Morphea: The 2023 update

JOURNAL=Frontiers in Medicine

VOLUME=Volume 10 - 2023

YEAR=2023

URL=https://www.frontiersin.org/journals/medicine/articles/10.3389/fmed.2023.1108623

DOI=10.3389/fmed.2023.1108623

ISSN=2296-858X

ABSTRACT=Morphea, also known as localized scleroderma, is a chronic inflammatory connective tissue disorder with variable clinical presentations, affecting both adults and children. It is characterized by inflammation and fibrosis of the skin and underlying soft tissue, in certain cases even the surrounding structures such as fascia, muscle, bone and central nervous system. While the etiology is still unknown, many factors may contribute to disease development, such as genetic predisposition, vascular dysregulation, TH1 / TH2 imbalance with chemokines and cytokines associated with interferon-γ and profibrotic pathways, as well as certain environmental factors. Since the disease may progress to permanent cosmetic and functional sequelae, it is crucial to properly assess the disease activity and to initiate promptly the adequate treatment, thus preventing subsequent damage. The mainstay of treatment is based on corticosteroids and methotrexate. These, however, are limited by their toxicity, especially if applied long-term. Furthermore, corticosteroids and methotrexate often do not sufficiently control disease, and/or the frequent relapses of morphea. This review presents the current understanding of morphea, by discussing its epidemiology, diagnosis, management and prognosis. In addition, it will describe recent findings in the complex pathogenesis of the disease, proposing potential novel targets for therapeutic development.