Morphea and Eosinophilic Fasciitis: An Update

Area of Science:

• Dermatology
• Rheumatology
• Autoimmune Diseases

Background:

• Morphea, or localized scleroderma, includes idiopathic sclerotic skin diseases with varying severity.
• Eosinophilic fasciitis (EF) is considered a severe form of morphea, sharing pathogenic processes with systemic sclerosis (SSc).
• Distinguishing morphea from SSc relies on the absence of specific clinical features like sclerodactyly and Raynaud's phenomenon.

Purpose of the Study:

• To provide a comprehensive overview of morphea and EF.
• To present a state-of-the-art treatment flowchart for morphea and EF.
• To highlight the importance of data harmonization and validated outcome measures like the LoSCAT.

Main Methods:

• Review of current literature on morphea and EF pathogenesis, diagnosis, and treatment.
• Development and presentation of a clinical management flowchart.
• Discussion of diagnostic criteria and monitoring tools, including the Localized Scleroderma Cutaneous Assessment Tool (LoSCAT).

Main Results:

• Morphea diagnosis is primarily clinical, while EF requires full-thickness skin biopsies.
• The LoSCAT is a validated outcome measure for morphea, aiding in disease monitoring.
• Treatment strategies vary from topical therapies for superficial subtypes to phototherapy or systemic agents like methotrexate for more severe cases.

Conclusions:

• Effective management of morphea and EF requires consideration of disease subtype, activity, depth, and quality of life.
• The presented flowchart guides clinicians in selecting appropriate treatments.
• Further research is needed to identify alternative treatments to methotrexate for these debilitating conditions.