表现为纵向厚甲的表皮错构瘤：一种新的甲部遗传性皮肤病。
Epidermal hamartoma presenting as longitudinal pachyleukonychia: a new nail genodermatosis.
作者信息
Moulin G, Baran R, Perrin C
机构信息
Dermatology Department, Hôpital de l'Antiquaille, Lyon.
出版信息
J Am Acad Dermatol. 1996 Nov;35(5 Pt 1):675-7. doi: 10.1016/s0190-9622(96)90719-7.
BACKGROUND
Epidermal hamartomas involving the nail apparatus are uncommon.
OBJECTIVE
Our purpose was to define the clinical, genetic, and histologic features of a unique nail abnormality.
METHODS
We examined eight members of three generations who presented with an unusual nail abnormality, longitudinal pachyleukonychia, as a sole characteristic that appeared between the ages of 9 and 30 years. One nail was examined histologically.
RESULTS
Histologic examination showed pronounced hyperplasia of the nail bed with papillomatosis. Cell nests of a sebaceous type were included in the epithelial digitations. This dysplasia was not accompanied by abnormalities affecting skin, hair, teeth, or sweat glands.
CONCLUSION
This unique epidermal hamartoma appears to be inherited as an autosomal dominant condition.
背景
累及甲器的表皮错构瘤并不常见。
目的
我们的目的是明确一种独特甲异常的临床、遗传和组织学特征。
方法
我们检查了三代人中的八名成员，他们均表现出一种不寻常的甲异常，即纵向厚甲白甲，这是他们9至30岁之间出现的唯一特征。对其中一个指甲进行了组织学检查。
结果
组织学检查显示甲床明显增生伴乳头瘤病。上皮指状突中包含皮脂腺型细胞巢。这种发育异常并未伴有影响皮肤、毛发、牙齿或汗腺的异常。
结论
这种独特的表皮错构瘤似乎作为常染色体显性疾病遗传。
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