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Unusual Idiopathic Calcinosis Cutis Universalis in a Child

机译:小儿不寻常的特发性Calcinosis Cutis Universalis

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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.
机译:皮肤角质炎是一种罕见的疾病,其特征在于磷酸钙(羟基磷灰石)晶体在人体各个部位的皮肤中逐渐沉积。根据病因将其分为四种类型,即钙和磷水平正常且存在组织损伤时为营养不良;钙和磷水平正常且不存在组织损伤时为特发性;或如果存在高钙血症或高钙血症则为转移性高磷酸盐血症。药物治疗和外科治疗是治疗皮肤角质病的选择。药物治疗不是很有效。手术切除已被证明是有益的,因为它可以减轻症状。但是,由于皮肤角质病的局限性并不总是很明确,因此可能会复发。我们在一个健康的6岁女孩中处理了一种非常罕见的皮肤角质病。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角质病,尽管特发性皮肤中的钙化最常见于一个区域,但我们的患者异常地表现出广泛的钙化沉积物。尽管现有的病灶显示出缓慢的改善,但全身性帕米膦酸治疗可有效预防新病灶的发生。手术切除被证明是一种有效而成功的治疗方法。该报告旨在提高医生对相对罕见的特发性钙化病皮肤的表现,病因和过程的认识。

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