Parry–Romberg syndrome associated with en coup de sabre in a patient from South Sudan – a rare entity from East Africa: a case report

A familial case of scleroderma en coup de sabre.

Sir, Linear scleroderma represents a rare disease, more often observed in the pediatric age group. The lower extremities are usually involved; less commonly it affects the frontoparietal area and the anterior scalp. Recently, a large case series of frontoparietal scleroderma en coup de sabre has been reported (1), but in this series, as well as in the English language literature, to the best of...

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...

Scleroderma 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.

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 ...

Can lesions of ‘en coup de sabre’ progress after being quiescent for a decade?