UMMS Affiliation
Department of Dermatology
Publication Date
2021-12-01
Document Type
Article
Disciplines
Dermatology | Immune System Diseases | Skin and Connective Tissue Diseases
Abstract
Morphea (localized scleroderma) is a rare autoimmune connective tissue disease with variable clinical presentations, with an annual incidence of 0.4-2.7 cases per 100,000. Morphea occurs most frequently in children aged 2-14 years, and the disease exhibits a female predominance. Insights into morphea pathogenesis are often extrapolated from studies of systemic sclerosis due to their similar skin histopathologic features; however, clinically they are two distinct diseases as evidenced by different demographics, clinical features, disease course and prognosis. An interplay between genetic factors, epigenetic modifications, immune and vascular dysfunction, along with environmental hits are considered as the main contributors to morphea pathogenesis. In this review, we describe potential new therapies for morphea based on both preclinical evidence and ongoing clinical trials. We focus on different classes of therapeutics, including antifibrotic, anti-inflammatory, cellular and gene therapy, and antisenolytic approaches, and how these target different aspects of disease pathogenesis.
Keywords
clinical trial, localized scleroderma, morphea, treatment
Rights and Permissions
© 2021 The Authors. This is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.
DOI of Published Version
10.1002/iid3.475
Source
Wenzel D, Haddadi NS, Afshari K, Richmond JM, Rashighi M. Upcoming treatments for morphea. Immun Inflamm Dis. 2021 Dec;9(4):1101-1145. doi: 10.1002/iid3.475. Epub 2021 Jul 17. PMID: 34272836; PMCID: PMC8589364. Link to article on publisher's site
Journal/Book/Conference Title
Immunity, inflammation and disease
Related Resources
PubMed ID
34272836
Repository Citation
Wenzel D, Haddadi N, Afshari K, Richmond JM, Rashighi M. (2021). Upcoming treatments for morphea. Open Access Publications by UMass Chan Authors. https://doi.org/10.1002/iid3.475. Retrieved from https://escholarship.umassmed.edu/oapubs/4841
Creative Commons License
This work is licensed under a Creative Commons Attribution 4.0 License.
Included in
Dermatology Commons, Immune System Diseases Commons, Skin and Connective Tissue Diseases Commons