Papillon-Lefèvre综合征1例  

A Case of Papillon-Lefèvre Syndrome

作　　者：郭建辉[1] 刘建[1] 郭雯[1] 赵丽[1] 刘玉华[1] 

机构地区：[1]河北医科大学沧州中西医结合临床医学院皮肤科,河北沧州061001

出　　处：《中国皮肤性病学杂志》2011年第3期221-222,共2页The Chinese Journal of Dermatovenereology

摘　　要：患者男,12岁。双手、双足、双肘和双膝部皮肤弥漫性角化10年。皮肤科情况:双手、双足、双肘和双膝部皮肤红斑角化,伴有鳞屑。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,牙排列不齐。左手背部皮损组织病理示: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:Papil-lon-Lefèvre综合征。A 12-year-old boy presented with skin diffuse cornification for ten years. Dermatological examination showed that diffuse comification on the hands, feet,elbows and knees, with scale. He was suffered from the gingivitis since the first years of life. Periodontium was damaged during the permanent teeth eruption. Oral cavity examination showed the flare of the gum and irregularity of teeth. The pathological examination（ left hand lesion） showed that psoriasiforul epidermal hyperplasia. Perivascular inflammatory cell infiltrate in the superficial dermis with papillary edema. The case was finally diagnosed as Papillon-Lefevre syndrome.

关 键 词：Papilhm—Lefevre综合征 常染色体隐性遗传 

分 类 号：R758.59[医药卫生—皮肤病学与性病学]