Bazopoulou-Kyrkanidou E, Tosios K I, Zabelis G, Charalampopoulou S, Papanicolaou S I
Division of Oral Pathology, Faculty of Dentistry, University of Athens, Greece.
J Oral Pathol Med. 1998 May;27(5):233-7. doi: 10.1111/j.1600-0714.1998.tb01948.x.
Hyalinosis cutis et mucosae (lipoid proteinosis, Urbach-Wiethe disease) is a rare syndrome with autosomal recessive inheritance. The disease is characterized by diffuse deposition of a hyaline-like substance in the dermis, the submucosal connective tissue and various internal organs. The oral mucosa of affected people becomes nodular and thickened, with primary involvement of the labial, buccal and palatal mucosa, posterior tongue, and lingual frenulum. We report the case of a 66-year-old man with hyalinosis cutis et mucosae who presented with gingival hyperplasia due to diffuse deposition of hyaline-like material, and discuss the diagnostic significance of the microscopic findings of the gingival tissues. The present clinical features are also compared to those of the same patient at the age of 38 years.
皮肤黏膜透明变性（类脂质蛋白沉积症，乌尔巴赫 - 维特病）是一种罕见的常染色体隐性遗传综合征。该疾病的特征是一种透明样物质在真皮、黏膜下结缔组织和各种内脏器官中弥漫性沉积。患者的口腔黏膜会出现结节状增厚，主要累及唇、颊、腭黏膜、舌后部和舌系带。我们报告了一例66岁患有皮肤黏膜透明变性的男性患者，其因透明样物质的弥漫性沉积出现牙龈增生，并讨论了牙龈组织显微镜检查结果的诊断意义。还将该患者目前的临床特征与其38岁时的情况进行了比较。
