Progressive Hemifacial Atrophy and Linear Scleroderma En Coup de Sabre: A Spectrum of the Same Disease?
نویسندگان
چکیده
منابع مشابه
Progressive Hemifacial Atrophy and Linear Scleroderma En Coup de Sabre: A Spectrum of the Same Disease?
Similar clinical and histhopathological features in progressive hemifacial atrophy and linear scleroderma en coup de sabre are well known. Trauma may predispose to the development of both diseases. The lack of association with anti-Borrelia antibodies was shown in both cases as well. The otolaryngological and endocrine disorders may be associated findings in both diseases. However, there are ce...
متن کاملLinear scleroderma en coup the sabre, progressive facial hemiatrophy and Rasmussen encephalitis : a single disease spectrum?
Results 9 patients presented with LSCS, followed in 5 of them by progressive hemifacial atrophy (PFH) within 2 years. 3 patients presented with PFH, all 3 had additional scleroderma lesions. Their median(range) age at presentation was 8(4-17)yrs. Extracutaneous manifestations were equally found in LSCS and PFH+LSCS patients. They comprised asymmetry of tooth arches/missing teeth(1), ophthalmolo...
متن کاملScleroderma en coup de sabre.
A case of scleroderma 'en coup de sabre' presenting with uveitis, predominantly posterior, and intractable grand mal seizures is described. The histopathology specimens and neuroradiology investigations reaffirm the clinical impression that inflammation is not solely confined to the skin in this form of linear scleroderma.
متن کاملAtypical Neuroimaging Manifestations of Linear Scleroderma “en coup de sabre”
Linear scleroderma "en coup de sabre" is a subset of localized scleroderma with band-like sclerotic lesions typically involving the fronto-parietal regions of the scalp. Patients often present with neurologic symptoms. On imaging, patients may have lesions in the cerebrum ipsilateral to the scalp abnormality. Infratentorial lesions and other lesions not closely associated with the overlying sca...
متن کاملLinear scleroderma en coup de sabre with associated neurologic abnormalities.
Linear scleroderma represents a unique form of localized scleroderma that primarily affects the pediatric population, with 67% of patients diagnosed before 18 years of age. When linear scleroderma occurs on the head, it is referred to as linear scleroderma en coup de sabre, given the resemblance of the skin lesions to the stroke of a sabre. Here we describe 3 pediatric patients with linear scle...
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ژورنال
عنوان ژورنال: Frontiers in Medicine
سال: 2018
ISSN: 2296-858X
DOI: 10.3389/fmed.2017.00258