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学科主题: 临床医学
题名:
Ultrastructural features of ichthyosis hystrix strongly resembling Lambert type
作者: Wang, W-H.1; Li, L-F.1; Zhang, Q.1; Yang, S-M.1; Jiang, W.1; Wang, Y-Y.1; Lei, P-C.1; Chen, X-R.1
关键词: binuclear cell ; ichthyosis hystrix ; shell ; tonofilament
刊名: BRITISH JOURNAL OF DERMATOLOGY
发表日期: 2007-05-01
DOI: 10.1111/j.1365-2133.2007.07792.x
卷: 156, 期:5, 页:1027-1031
收录类别: SCI
文章类型: Article
WOS标题词: Science & Technology
类目[WOS]: Dermatology
研究领域[WOS]: Dermatology
关键词[WOS]: CURTH-MACKLIN ; EPIDERMOLYTIC HYPERKERATOSIS ; MUTATION ; HETEROGENEITY ; ABNORMALITIES ; GRAVIOR ; FAMILY ; LIGHT
英文摘要:

Ichthyosis hystrix (IH) is characterized by spiny hyperkeratotic scale, and includes Brocq type, Lambert type, Curth-Macklin type, Rheydt type and Bafverstedt type. The first documented cases of familial IH were of Lambert type. However, the ultrastructural features of IH Lambert type have not been reported. Three patients in two generations of a family from north China were observed. The patients showed widespread verrucose lesions without blister formation. The face, palms and soles were unaffected. This presentation strongly resembled IH Lambert type. The lesions faded dramatically in summer, without treatment. Light microscopic examinations showed binuclear cells and shell formation in the granular and upper spinous layers in all specimens, with similar findings in winter, when lesions were prominent, and in summer, when lesions had subsided. Electron microscopic examination revealed binuclear keratinocytes and concentric, thin to thick, unbroken shells of tonofilaments surrounding the nuclei, and segregation of cytoplasmic components. This family is the first with familial IH strongly resembling Lambert type to be reported in China. Binuclear cells and tonofilaments shells surrounding the nucleus in upper keratinocytes were characteristic features, which were similar to those reported in IH Curth-Macklin type. The basic histopathological defects were not changed despite significant clinical improvement of the lesion.

语种: 英语
WOS记录号: WOS:000245938700033
Citation statistics:
内容类型: 期刊论文
URI标识: http://ir.bjmu.edu.cn/handle/400002259/49851
Appears in Collections:北京大学第三临床医学院_皮肤科_期刊论文

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作者单位: 1.Peking Univ, Hosp 3, Dept Dermatol, Beijing 100083, Peoples R China
2.Peking Univ, Dept Pathol, Beijing 100083, Peoples R China

Recommended Citation:
Wang, W-H.,Li, L-F.,Zhang, Q.,et al. Ultrastructural features of ichthyosis hystrix strongly resembling Lambert type[J]. BRITISH JOURNAL OF DERMATOLOGY,2007,156(5):1027-1031.
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