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Neurologic Involvement in Scleroderma en Coup de Sabre

DOI: 10.1155/2012/719685

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Localized scleroderma is a rare disease, characterized by sclerotic lesions. A variety of presentations have been described, with different clinical characteristics and specific prognosis. In scleroderma en coup de sabre (LScs) the atrophic lesion in frontoparietal area is the disease hallmark. Skin and subcutaneous are the mainly affected tissues, but case reports of muscle, cartilage, and bone involvement are frequent. These cases pose a difficult differential diagnosis with Parry-Romberg syndrome. Once considered an exclusive cutaneous disorder, the neurologic involvement present in LScs has been described in several case reports. Seizures are most frequently observed, but focal neurologic deficits, movement disorders, trigeminal neuralgia, and mimics of hemiplegic migraines have been reported. Computed tomography and magnetic resonance imaging have aided the characterization of central nervous system lesions, and cerebral angiograms have pointed to vasculitis as a part of disease pathogenesis. In this paper we describe the clinical and radiologic aspects of neurologic involvement in LScs. 1. Introduction Scleroderma is a rare disease of unknown etiology, characterized by thickening and hardening of skin resulting from increased collagen production. The term includes a variety of diseases, from localized scleroderma (LS) to systemic sclerosis. LS is traditionally considered to be limited to skin, subcutaneous tissue, underlying bone, and, in craniofacial subtype, nervous system involvement [1]. Recent studies, however, have described malaise, fatigue, arthralgia, and myalgia in morphea. Moreover, rheumatologic, ophthalmologic and neurologic symptoms and signs have been described in up to 20% of the patients with LS. Based on these findings LS ought to be differentiated from systemic sclerosis by the absence of sclerodactylya, Raynaud’s phenomenon, and capillaroscopic abnormalities [1]. LS incidence ranges from 0.4 to 2.7 per 100,000 people [2]. Although present in all races, the prevalence among Caucasians is increased, summing up 72 to 82% of the patients [2]. Females are primarily affected [1], and a similar distribution between children and adults occurs [1, 3]. Disease incidence peaks in the fifth decade of life in adults, whereas 90% of children are diagnosed between 2 and 14 years of age [1, 3–5]. Linear scleroderma en coup de sabre (LCsc) is a rare subset of LS. The typical presentation affects frontoparietal region, and the mean age of onset is around 13 years old [1]. In this paper, clinical presentation of LScs and its neurological


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