Primary Complete Pachydermoperiostosis: A Case Report

Pachydermoperiostosis, also known as Touraine-Solente-Gole syndrome, is a primary hypertrophic osteoarthropathy that is inherited as an autosomal dominant trait with variable expression. This rare disease usually begins insidiously at puberty and is more common in men than in women. A 35 year-old man with pachydermoperiostosis was presented. He presented with leonine appearance, bilateral ptosis, cutis verticis gyrata and clubbing in the distal phalanges. Plain radiograph of hand showed periostosis.

Primer Komplet Pakidermoperiostoz: Olgu Sunumu

Touraine-Solente-Gole sendromu olarak da bilinen pakidermoperiostozis (PDP) deðiþken geçiþli otozomal dominant kalýtým gösteren bir primer hipertrofik osteoartropatidir. Bu nadir hastalýk genellikle puberte döneminde sinsi olarak baþlar ve erkeklerde kadýnlardan daha yaygýndýr. 35 yaþýndaki erkek hastamýzda aslan yüzü görünümü, her iki göz kapaðýnda ptozis, kutis vertisis girata, distal falankslarda çomaklaþma gözlendi. El direk grafisinde periostozis görüldü.