掌跖角化-牙周破坏综合征1例

One case report of papillon-Lefèvre syndrome

患者男,13岁。牙齿松动和双手、双足、双肘、双膝部皮肤弥漫性角化9年。自幼牙龈红肿化脓,恒牙萌出时牙周组织出现破坏,牙龈红肿,牙齿排列不齐。左手背部皮损组织病理检查示:表皮银屑病样增生,真皮浅层水肿,少许慢性炎细胞浸润。诊断:Papillon-Lefèvre综合征。

A 13-year-old boy presented with loose teeth and skin diffuse cornification for nine years.He has been suffering from the gingivitis since the first year of life.Periodontium was damaged during the permanent teeth eruption.Oral cavity examinntion showed the flare of the gum and irregularity of teeth.The pathological examination of the left hand lesion showed psoriasiform epidermal hyperplasia,perivascular inflammatory cell infiltration in the superficial dermis with papillary edema.The case was finally diagnosed as Papillon-Lefèvre syndrome.