Olmsted syndrome-palmoplantar and periorificial keratodermas: Association with malignant melanoma

Olmsted syndrome-palmoplantar and periorificial keratodermas: Association with malignant melanoma
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DOI:
10.1007/s10227-002-0107-4
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发表时间:
2003-05-01
影响因子:
2.3
通讯作者:
Gagne, E
Gagne, E
中科院分区:
医学4区
文献类型:
--
作者:
Dessureault, J;Poulin, Y;Gagne, E

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背景资料:Olmsted综合征是一种罕见的先天性疾病,残害掌跖角化病,周围角化斑块,和其他变量footprints.Objective:我们描述了一个65岁的女子与Olmsted综合征并发的恶性黑色素瘤内的足底角化病的发生。据我们所知,这是第一次报告的情况下,这样的发生在奥姆斯特德综合征。这种罕见的disorder.Conclusion:恶性上皮肿瘤和Olmsted综合征之间的关联已被报道。恶性黑色素瘤与其他类型的掌跖角化病的关系已有报道。这可能提示先天性角化病患者易患黑色素细胞和鳞状细胞恶性肿瘤。口服类维生素A似乎是最有希望的治疗奥姆斯特德综合征和其他症状性角化病。
Background: Olmsted syndrome is a rare congenital disorder with mutilating palmoplantar keratoderma, periorificial keratotic plaques, and other variable features.Objective: We describe a 65-year-old woman with Olmsted syndrome complicated by the occurrence of a malignant melanoma inside the plantar keratoderma. To our knowledge, this is the first reported case of such an occurrence in Olmsted syndrome. The published cases of this rare disorder are reviewed.Conclusion: An association between malignant epithelial tumors and Olmsted syndrome has already been reported. The association of malignant melanoma with other types of palmoplantar keratodermas has been reported. This may suggest a predisposition to melanocytic as well as squamous cell malignancies in congenital keratodermas. Oral retinoids appear to be the most promising treatment for Olmsted syndrome and for other symptomatic keratodermas.