how to cite this article: allmendinger am, ricci ja, desai ns, viswanadhan n, rodriguez d. atypical neuroimaging manifestations of linear scleroderma “en coup de sabre”. iran j child neurol. summer 2015;9(3):62-68. abstract 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. pat...

Linear scleroderma is a form of localized scleroderma that primarily affects the pediatric population. When it occurs on the scalp or forehead, it is termed "en coup de sabre". In the en coup de sabre subtype, many extracutaneous associations, mostly neurological, have been described. A patient with linear scleroderma en coup de sabre was noted to have ipsilateral brain cavernomas by magnetic r...

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...

Parry-Romberg syndrome (PRS) is a relatively rare degenerative disorder that is poorly understood. PRS is characterized by slowly progressing atrophy affecting one side of the face, and is frequently associated with localized scleroderma, especially linear scleroderma, which is known as en coup de sabre. This is a report of the author's experiences with PRS accompanying en coup de sabre, and a ...

BACKGROUND Hemifacial atrophy (Parry-Romberg syndrome) is a relatively rare disease. The etiology of the disease is not clear. Some authors postulate its relation with limited scleroderma linearis. Linear scleroderma "en coup de sabre" is characterized by clinical presence of most commonly one-sided linear syndrome. In a number of patients, neurological affection is the medium of the disease. T...

Linear morphea in an en coup de sabre pattern is a variant of localized scleroderma that develops on the frontal or frontoparietal scalp. Approximately two-thirds of cases occur before the age of 18. The etiology is unknown, but the disease is thought to be most likely autoimmune in nature. Between 50% and 75% of these patients have been found to have a positive serum antinuclear antibody (ANA)...

Extracutaneous calcifications are rare in scleroderma and related conditions. We describe a female patient with linear scleroderma en coup de sabre and a longstanding clinical history of tonic and clonic convulsions. Radiographic study showed extensive cerebral calcifications in the right occipital hemisphere, homolateral to the involved side of her face. This report further suggests a relation...

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.

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...

A case is reported in which an 11-year-old girl developed progressive ptosis and a subsequent motility disorder of the right eye. The diagnosis linear scleroderma en coup de sabre was established. Atrophy of the upper levator palpebral and superior rectus muscle could be shown on CT scan.