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掌跖角化病Unna - Thost型与Vörner型的鉴别指征。110年后对托斯特家族的重新审视。

Indication for the identity of palmoplantar keratoderma type Unna-Thost with type Vörner. Thost's family revisited 110 years later.

作者信息

Küster W, Becker A

机构信息

Department of Dermatology, University of Düsseldorf, Germany.

出版信息

Acta Derm Venereol. 1992;72(2):120-2.

PMID:1350396
Abstract

Palmoplantar keratoderma (PPK) type Unna-Thost is known to be the most common form of a hereditary disorder of keratinization of palms and soles. The disease is clinically identical with PPK type Vörner which is histologically characterized by epidermolytic hyperkeratosis. By reinvestigation of the family originally seen by Thost in 1880, the features of epidermolytic hyperkeratosis were found histologically confirming the diagnosis PPK of Vörner. This proves the identity of PPK type Thost with PPK of Vörner. Because of the histological variability of epidermolytic hyperkeratosis, detailed light and electron microscopic studies are necessary in cases of diffuse types of PPK.

摘要

掌跖角化病(PPK)Unna-Thost型是已知最常见的遗传性掌跖角化障碍形式。该疾病在临床上与Vörner型PPK相同,其组织学特征为表皮松解性角化过度。通过对Thost于1880年首次观察的家族进行重新研究,在组织学上发现了表皮松解性角化过度的特征,从而证实了Vörner型PPK的诊断。这证明了Thost型PPK与Vörner型PPK的一致性。由于表皮松解性角化过度存在组织学变异性,对于弥漫型PPK病例,需要进行详细的光镜和电镜研究。

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