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A practical clinical approach to the diagnosis and management of morphea (localized scleroderma)
Caterina Matucci-Cerinic1, Dan Lipsker2, Clara Malattia1
1UOC Reumatologia e Malattie Autoinfiammatorie, IRCCS Istituto Giannina Gaslini, Genova, Italy; Dipartimento di Neuroscienze, Riabilitazione, Oftalmologia, Genetica e Scienze Materno-infantili - DINOGMI, Università Degli Studi di Genova, Genova, Italy.
Abstract:
Morphea, also referred to as localized cutaneous scleroderma, encompasses a heterogeneous group of inflammatory and fibrosing skin disorders characterized by variable depth of tissue involvement and different clinical course. The diagnostic delay remains common due to insidious onset, broad differential diagnosis, and lack of validated biomarkers of disease activity. Clinical assessment relies therefore on expert evaluation, supported by clinical scoring systems and selected imaging techniques. Therapeutic strategies depend on disease subtype, activity, depth, and risk of irreversible damage, ranging from topical agents for limited superficial forms to systemic immunosuppression for deep, linear, generalized, and pansclerotic variants. Methotrexate combined with systemic corticosteroids represents the current first-line systemic therapy, while mycophenolate mofetil and biologics or targeted synthetic agents are increasingly used in refractory disease. This review provides a comprehensive and updated overview of the epidemiology, clinical spectrum, diagnostic approach, and management of morphea across age groups, highlighting recent therapeutic advances, and unmet clinical needs.
Insights
Morphea, a localized scleroderma, presents diverse skin issues. Early diagnosis is challenging, but current treatments, including methotrexate and corticosteroids, manage symptoms effectively, with newer options for resistant cases.
Area of Science:
- Dermatology
- Rheumatology
- Immunology
Background:
- Morphea, or localized cutaneous scleroderma, is a complex inflammatory and fibrosing skin condition.
- It exhibits varied tissue involvement and clinical progression, often leading to diagnostic delays due to subtle onset and lack of specific biomarkers.
Purpose of the Study:
- To provide a comprehensive review of morphea's epidemiology, clinical presentation, diagnostic methods, and management strategies.
- To highlight recent therapeutic advancements and identify unmet clinical needs in morphea treatment across all age groups.
Main Methods:
- Literature review of epidemiological data, clinical characteristics, diagnostic tools (clinical evaluation, scoring systems, imaging), and therapeutic approaches.
- Analysis of current first-line (methotrexate, corticosteroids) and advanced therapies (mycophenolate mofetil, biologics) for different morphea subtypes.
Main Results:
- Morphea diagnosis is often delayed, relying on expert clinical assessment and imaging.
- Treatment is tailored to disease subtype, activity, depth, and damage risk, with established and emerging therapeutic options.
Conclusions:
- Morphea management requires a multidisciplinary approach, balancing established therapies with newer agents for refractory cases.
- Further research is needed to improve diagnostic accuracy and address unmet needs in morphea treatment.
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