ABSTRACT Localized scleroderma (also called morphea) is a term encompassing a spectrum of sclerotic autoimmune diseases that primarily affect the skin, but also might involve underlying structures such as the fat, fascia, muscle, and bones. Its exact pathogenesis is still unknown, but several trigger factors in genetically predisposed individuals might initially lead to an immunologically triggered release of pro-inflammatory cytokines, resulting in a profound dysregulation of the connective tissue metabolism and ultimately to induction of fibrosis. To date, there are no specific serological markers available for localized scleroderma. Within the last years, several validated clinical scores have been introduced as potential outcome measures for the disease. Given the rarity of localized scleroderma, only few evidence-based therapeutical treatment options exist. So far, the most robust data is available for ultraviolet A1 phototherapy in disease that is restricted to the skin, and methotrexate alone or in combination with systemic corticosteroids in more severe disease that additionally affects extracutaneous structures. This practical review summarizes relevant information on the epidemiology, pathogenesis, clinical subtypes and classifications, differential diagnoses, clinical scores and outcome measures, and current treatment strategies of localized scleroderma.
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ABSTRACT: Localized scleroderma is traditionally considered to be limited to the skin, subcutaneous tissue, underlying bone, and in the craniofacial subtype, also nervous system involvement. However, recent studies have also described other systemic manifestations in these patients. Despite many reports of neurological involvement in patients with the craniofacial linear localized scleroderma, it is extremely rare in patients with the other subtypes of localized scleroderma. Here, we report an extraordinary case of localized scleroderma en plaque (classic morphea), located to the upper trunk and neck, associated with neurological manifestations presented as seizures. Magnetic resonance imaging of the brain showed focal lesions on the contralateral side to the skin involvement. This case is extremely relevant not only due to its rarity, but also because it supports the idea that the pathogenesis of the localized scleroderma is related to a systemic autoimmune process. • Localized scleroderma is known to be related with systemic symptoms. • To the best of our knowledge, this is the first case report of an exclusive plaque morphea with neurological involvement. • This case provides support to the concept that the pathogenesis of the disease is a systemic autoimmune process.Clinical Rheumatology 12/2013; DOI:10.1007/s10067-013-2458-1 · 1.77 Impact Factor
01/2013; 16:114. DOI:10.11604/pamj.2013.16.114.3065
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ABSTRACT: Morphea (localized scleroderma) is a rare fibrosing disorder of the skin and underlying tissues characterized by skin thickening and hardening due to increased collagen deposition. The significance of the disease depends on the extent of the lesions, potential for functional disability or cosmetic disfigurement, and presence or absence of extracutaneous manifestations. Treatment is tailored; circumscribed forms may require only topical treatment, while forms causing functional impairment or severe cosmetic change may require systemic treatment. Although localized scleroderma has a good prognosis, some clinical subtypes can be deforming and irreversibly disabling, especially when affecting the extremities or the face.Pediatric Clinics of North America 04/2014; 61(2):309-319. DOI:10.1016/j.pcl.2013.11.006 · 2.20 Impact Factor