BACKGROUND: Morphea is a rare chronic inflammatory disease that involves skin and subcutaneous tissues, causing skin sclerosis. Generalized morphea is characterized by extensive cutaneous involvement and poor response to therapy. Although the pathophysiology for morphea is not completely understood, abnormal signaling through the platelet-derived growth factor and transforming growth factor beta axes seems to play a major role in the inflammatory response and sclerotic process. Imatinib, a tyrosine kinase inhibitor, interferes with both transforming growth factor beta and platelet-derived growth factor signaling pathways. Recent studies proved imatinib's efficacy in the prevention and regression of fibrosis associated with systemic sclerosis, nephrogenic sclerosis, and bleomycin-related fibrosis. OBSERVATIONS: We present the case of a 50-year-old Caucasian man with a generalized morphea diagnosed 10 years ago, with multiple ulcers, who was treated with imatinib over the course of 12 months. At the end of the treatment, most of the skin ulcerations had healed, and cutaneous thickness was reduced as demonstrated by skin biopsy and ultrasound evaluation. The patient also experienced an improvement in articular mobility, sustained by a 20° increase in left knee extension. CONCLUSIONS: Although controlled studies are necessary to access the antifibrotic effect of imatinib in morphea, the present report shows its potential role in the treatment of this condition.
BACKGROUND: Morphea is a rare chronic inflammatory disease that involves skin and subcutaneous tissues, causing skin sclerosis. Generalized morphea is characterized by extensive cutaneous involvement and poor response to therapy. Although the pathophysiology for morphea is not completely understood, abnormal signaling through the platelet-derived growth factor and transforming growth factor beta axes seems to play a major role in the inflammatory response and sclerotic process. Imatinib, a tyrosine kinase inhibitor, interferes with both transforming growth factor beta and platelet-derived growth factor signaling pathways. Recent studies proved imatinib's efficacy in the prevention and regression of fibrosis associated with systemic sclerosis, nephrogenic sclerosis, and bleomycin-related fibrosis. OBSERVATIONS: We present the case of a 50-year-old Caucasian man with a generalized morphea diagnosed 10 years ago, with multiple ulcers, who was treated with imatinib over the course of 12 months. At the end of the treatment, most of the skin ulcerations had healed, and cutaneous thickness was reduced as demonstrated by skin biopsy and ultrasound evaluation. The patient also experienced an improvement in articular mobility, sustained by a 20° increase in left knee extension. CONCLUSIONS: Although controlled studies are necessary to access the antifibrotic effect of imatinib in morphea, the present report shows its potential role in the treatment of this condition.
Authors: Jorre S Mertens; Marieke M B Seyger; Rogier M Thurlings; Timothy R D J Radstake; Elke M G J de Jong Journal: Am J Clin Dermatol Date: 2017-08 Impact factor: 7.403