Fiche publication
Date publication
mars 2026
Journal
Best practice & research. Clinical rheumatology
Auteurs
Membres identifiés du Cancéropôle Est :
Pr LIPSKER Dan
Tous les auteurs :
Matucci-Cerinic C, Lipsker D, Malattia C, Martini G
Lien Pubmed
Résumé
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.
Mots clés
Coup-de-sabre, Localized cutaneous scleroderma, Morphea, Pansclerotic, Parry-romberg syndrome
Référence
Best Pract Res Clin Rheumatol. 2026 03 20;:102127