Linear Scleroderma En Coup De Sabre

Abstract

We studied 58 cases of linear scleroderma of the face: 20 of them showed transition into progressive facial hemiatrophy (PFH). In several cases a distinction between these two conditions was not possible since the atrophic changes were arranged along the previous indurations, usually along the branches of trigeminal nerve. Neurological manifestations, most often epilepsy, were found in about 10% cases of PFH, and were similar in childhood cases of scleroderma en coup de sabre. The shrinking of deeper tissue and bone deformities were observed by early onset of the disease, not infrequently after trauma. In both scleroderma en coup de sabre and PFH, neurological complications are probably related to vascular involvement.