Scleroderma en coup de sabre.

Linear 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...

Brain cavernomas associated with en coup de sabre linear scleroderma: Two case reports

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...

Parry-Romberg Syndrome with En Coup de Sabre

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 ...

Linear scleroderma en coup de sabre and brain calcification: is there a pathogenic relationship?

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...

Linear scleroderma en coup de sabre and brain calcification: is there a pathogenic