副肿瘤性天疱疮2例

Paraneoplastic Pemphigus:A Report of Two Cases

例 1男 ,3 5岁 ,发病 6个月 ,有口腔、脸结膜及生殖器黏膜糜烂 ,躯干大片的红斑、水疱 ,尼氏征阳性。腹部CT示腹膜后肿瘤。患者因感染重 ,全身衰竭死亡。例 2男 ,5 2岁 ,头面部、躯干大片红斑、水疱 3个月 ,尼氏征阳性。部分区域有糜烂、结痂。外科剖腹探查发现回盲部肿瘤 。

Paraneoplastic pemphigus(PNP) is a distinct autoimmune bullous diseases,is not often seen.We here report two cases,their clinical,histologic,and immunopathologic findings are strongly suggested the diagnosis.