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一名儿童的罕见特发性泛发性皮肤钙化病

Unusual Idiopathic Calcinosis Cutis Universalis in a Child.

作者信息

Alabaz Derya, Mungan Neslihan, Turgut Mehmet, Dalay Cemil

机构信息

Department of Pediatric Infectious Diseases, Cukurova University Faculty of Medicine, Balcali, Turkey.

出版信息

Case Rep Dermatol. 2009 Aug 28;1(1):16-22. doi: 10.1159/000227285.

DOI:10.1159/000227285
PMID:20652108
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC2895204/
Abstract

Calcinosis cutis is an uncommon disorder characterized by the progressive deposition of crystals of calcium phosphate (hydroxyapatite) in the skin in various areas of the body. It is classified into four types according to etiology, namely as dystrophic if calcium and phosphorus levels are normal and tissue damage is present, as idiopathic if calcium and phosphorus levels are normal and no tissue damage is present, or as metastatic if there is hypercalcemia or hyperphosphatemia. Medical and surgical treatments are options to cure calcinosis cutis. Medical therapy is not very effective. Surgical excision has shown to be beneficial, as it can provide a symptomatic relief. However, since calcinosis cutis limits are not always well defined, a recurrence of the lesions may occur. We dealt with a very rare form of calcinosis cutis in a healthy 6-year-old girl. There was no evidence of connective tissue disorder or abnormal mineral metabolism. Hence, she was diagnosed as idiopathic calcinosis cutis and, although calcifications in idiopathic cutis are most commonly localized to one area, our patient unusually exhibited widespread calcific deposits. Although the existing lesions showed slow improvement, systemic pamidronate therapy was effective in preventing the occurrence of new lesions. Surgical excision proved to be an effective and successful treatment. This report aims to raise doctors' awareness on the presentation, etiopathogenesis, and course of the relatively rare idiopathic calcinosis cutis.

摘要

皮肤钙化症是一种罕见的疾病,其特征是磷酸钙(羟基磷灰石)晶体在身体各个部位的皮肤中进行性沉积。根据病因,它可分为四种类型,即如果钙和磷水平正常且存在组织损伤则为营养不良型,如果钙和磷水平正常且不存在组织损伤则为特发性,如果存在高钙血症或高磷血症则为转移性。药物和手术治疗是治疗皮肤钙化症的选择。药物治疗效果不太显著。手术切除已显示出有益效果,因为它可以缓解症状。然而,由于皮肤钙化症的边界并不总是很明确,病变可能会复发。我们处理了一名健康6岁女孩身上一种非常罕见的皮肤钙化症。没有结缔组织疾病或矿物质代谢异常的证据。因此,她被诊断为特发性皮肤钙化症,尽管特发性皮肤钙化症的钙化最常见于一个部位,但我们的患者异常地出现了广泛的钙化沉积。尽管现有病变显示出缓慢改善,但全身性帕米膦酸治疗在预防新病变的发生方面是有效的。手术切除被证明是一种有效且成功成功的治疗方法。本报告旨在提高医生对相对罕见的特发性皮肤钙化症的表现、病因发病机制和病程的认识。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6721/2895204/cbd81aa11ed5/cde0001-0016-f03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6721/2895204/56bbc960c585/cde0001-0016-f01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6721/2895204/f7513a05d608/cde0001-0016-f02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6721/2895204/cbd81aa11ed5/cde0001-0016-f03.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6721/2895204/56bbc960c585/cde0001-0016-f01.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6721/2895204/f7513a05d608/cde0001-0016-f02.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/6721/2895204/cbd81aa11ed5/cde0001-0016-f03.jpg

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本文引用的文献

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Bisphosphonates: mode of action and pharmacology.双膦酸盐:作用机制与药理学
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Calcinosis cutis universalis in a patient with systemic lupus erythematosus.系统性红斑狼疮患者的泛发性皮肤钙质沉着症。
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