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Early development of multiple epithelial neoplasms in Netherton syndrome.

作者信息

Krasagakis K, Ioannidou D J, Stephanidou M, Manios A, Panayiotides J G, Tosca A D

机构信息

Department of Dermatology, University General Hospital of Heraklion, Heraklion, Greece.

出版信息

Dermatology. 2003;207(2):182-4. doi: 10.1159/000071791.

DOI:10.1159/000071791
PMID:12920370
Abstract

We report a case of Netherton syndrome manifested as congenital ichthyosiform erythroderma, trichorrhexis invaginata and atopy, who in early adulthood developed multiple, aggressive epithelial neoplasms in sun-exposed areas of the skin, in areas with papillomatous skin hyperplasia and at the left parotid region. The occurrence of cutaneous neoplasia has been reported in syndromes with congenital ichthyosis and suggests that the underlying genetic defects may cause the development of cancer in prone patients.

摘要

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Comèl-Netherton syndrome defined as primary immunodeficiency.科梅耳-内瑟顿综合征被定义为原发性免疫缺陷。
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