Understanding Morphea: Insights into Localized Scleroderma

Morphea, or localized scleroderma, is a rare inflammatory connective tissue disorder characterized by skin thickening and fibrosis. This review provides an overview of morphea, including its epidemiology, pathogenesis, clinical types, diagnosis, and treatment options. Morphea predominantly affects children and women, with peaks in early childhood and the fifth decade of life. The disease involves genetic, environmental, and immune factors, leading to increased collagen production. Clinical forms include limited, generalized, linear, mixed, and pansclerotic types. Diagnosis is clinical, supported by histopathology and imaging. Treatment is individualized, involving topical therapies (corticosteroids, tacrolimus), systemic therapies (methotrexate, corticosteroids), and phototherapy (UVA, PUVA). Emerging therapies targeting specific pathways show promise. In children, early aggressive treatment is crucial to prevent complications. Postirradiation morphea and variants like keloid morphea require accurate diagnosis for appropriate management. Phototherapy, particularly low-dose UVA, is effective. Early diagnosis and tailored treatment are essential for managing morphea and preventing long-term complications.

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Publication Details

Journal
Zenodo (CERN European Organization for Nuclear Research)
Published
2024-09-26
DOI
https://doi.org/10.5281/zenodo.14052761
Primary Topic
Systemic Sclerosis and Related Diseases
Type
article
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article

Understanding Morphea: Insights into Localized Scleroderma

Zenodo (CERN European Organization for Nuclear Research)
Systemic Sclerosis and Related Diseases
article

Understanding Morphea: Insights into Localized Scleroderma

article en

Abstract

Morphea, or localized scleroderma, is a rare inflammatory connective tissue disorder characterized by skin thickening and fibrosis. This review provides an overview of morphea, including its epidemiology, pathogenesis, clinical types, diagnosis, and treatment options. Morphea predominantly affects children and women, with peaks in early childhood and the fifth decade of life. The disease involves genetic, environmental, and immune factors, leading to increased collagen production. Clinical forms include limited, generalized, linear, mixed, and pansclerotic types. Diagnosis is clinical, supported by histopathology and imaging. Treatment is individualized, involving topical therapies (corticosteroids, tacrolimus), systemic therapies (methotrexate, corticosteroids), and phototherapy (UVA, PUVA). Emerging therapies targeting specific pathways show promise. In children, early aggressive treatment is crucial to prevent complications. Postirradiation morphea and variants like keloid morphea require accurate diagnosis for appropriate management. Phototherapy, particularly low-dose UVA, is effective. Early diagnosis and tailored treatment are essential for managing morphea and preventing long-term complications.

Zenodo (CERN European Organization for Nuclear Research)
Silchar Medical College and Hospital (IN)
Good health and well-being
Openalex Percentile: Top 53%
Systemic Sclerosis and Related Diseases
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