Chapman M S, Perry A E, Baughman R D
Department of Medicine, Dartmouth Hitchcock Medical Center, Lebanon, New Hampshire 03756, USA.
Am J Dermatopathol. 1998 Aug;20(4):413-6. doi: 10.1097/00000372-199808000-00017.
The authors report a case of Lhermitte-Duclos disease, or dysplastic cerebellar gangliocytoma, in which a cutaneous sclerotic fibroma was found incidentally during the second resection of a recurrent cerebellar hamartoma. The association of Lhermitte-Duclos disease and sclerotic fibroma with Cowden's syndrome led to a dermatologic examination and confirmation of the diagnosis of Cowden's syndrome. The combination of both Lhermitte-Duclos disease and sclerotic fibroma with Cowden's syndrome has not previously been reported. A review of the 15 cases of Lhermitte-Duclos disease associated with Cowden's syndrome shows no significant gender predilection. Sclerotic fibromas, once thought to be specific for Cowden's syndrome, also are reviewed.
作者报告了一例Lhermitte-Duclos病，即发育异常性小脑神经节细胞瘤，其中在复发性小脑错构瘤的第二次切除术中偶然发现了皮肤硬化性纤维瘤。Lhermitte-Duclos病和硬化性纤维瘤与考登综合征的关联促使进行了皮肤科检查并确诊为考登综合征。此前尚未报道过Lhermitte-Duclos病和硬化性纤维瘤与考登综合征同时存在的情况。对15例与考登综合征相关的Lhermitte-Duclos病病例的回顾显示，无明显性别倾向。曾被认为是考登综合征特异性表现的硬化性纤维瘤也进行了回顾。
