白色海绵状斑痣ppt15共享精品课件

白色海绵状斑痣 White sponge nevus white sponge nevus n白色皱折病(White folded disease) n1935年由Cannon首先描述。在一个家庭 的 3 个成员均表现为口腔、阴道及直肠粘膜 呈特殊海绵状的白色或珠光色特征，他建议 称为白色海绵痣。 n常染色体显性遗传病 white sponge nevus n本病好发于颊、口底及舌腹粘膜，腭、 龈粘膜也可发病。患者无自觉症状，粘膜为 珍珠样白色，有皱折，触诊质软似海绵，不 似白斑表面发硬粗糙。病变从婴幼儿期即可 出现，到青春期达到高峰，以后不再发展。 除口腔病变外，阴唇、阴道、肛门及直肠等 处粘膜也可发生同样病变，本病不是癌前病 变，不发生恶性变。 white sponge nevus white sponge nevus white sponge nevus white sponge nevus white sponge nevus 【病理】 上皮明显增厚，基底细胞增多，但细 胞分化较好。棘层变化明显，细胞增大，层 次增多，甚至达40-50层以上。有些棘细胞 核周围有一透亮的环，这些细胞经PAS染色 及电镜证实富含糖原。有些棘细胞有空泡性 变，胞核固缩或消失；表层为增厚的、未脱 落的不全角化细胞，上皮表面高低不平；上 皮钉突增宽且有时融合。结缔组织中胶原纤 维水肿，断裂，有少量炎细胞浸润。 white sponge nevus white sponge nevus white sponge nevus white sponge nevus white sponge nevus white sponge nevus white sponge nevus white sponge nevus white sponge nevus white sponge nevus n 近年来研究表明，K4 及 K13 基 因发生突变，导致上皮（或表皮）棘层 细胞内角蛋白丝断裂，并聚集在细胞核 周围，从而引起本病的发生。 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