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

Link to Article in PubMed

PubMed ID

34272836

Creative Commons License

Creative Commons Attribution 4.0 License
This work is licensed under a Creative Commons Attribution 4.0 License.

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