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格赖特型掌跖角化病是一种独立的疾病实体吗?

Is palmoplantar keratoderma of Greither's type a separate nosologic entity?

作者信息

Kansky A, Arzensek J

出版信息

Dermatologica. 1979;158(4):244-8. doi: 10.1159/000250764.

DOI:10.1159/000250764
PMID:155015
Abstract

17 patients with palmoplantar keratoderma (PPK) were detected among 48 investigated members of the clan O. 5 of these 17 patients had a well-expressed hyperkeratosis on their knees. In one family hyperkeratosis of the knees was inherited as a dominant feature, but in two other families it was not. The observation was made that the expressivity of the disease was fading: while there were 15 PPK patients among the 25 investigated members in the generations II and III, there were only 2 patients among 22 members in the generations IV and V. In addition to PPK incontinentia pigmenti was diagnosed in two instances and pollex duplex in one. The question of the identity of PPK of Greither's type is shortly discussed.

摘要

在对O氏族的48名成员进行调查时,发现了17例掌跖角化病(PPK)患者。这17名患者中有5例膝盖处有明显的角化过度。在一个家族中,膝盖角化过度作为显性特征遗传,但在另外两个家族中并非如此。观察到该病的表现度正在减弱:在第二代和第三代的25名被调查成员中有15例PPK患者,而在第四代和第五代的22名成员中只有2例患者。此外,还诊断出2例色素失禁症和1例复拇畸形。简要讨论了Greither型PPK的同一性问题。

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