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[一例特发性多发性皮肤钙化症]

[A case of idiopathic multiple calcinosis cutis].

作者信息

Iannello S, Camuto M, Cavaleri A, Spina S, Belfiore F

机构信息

Cattedra di Medicina Interna, Università degli Studi, Catania Ospedale Garibaldi.

出版信息

Minerva Med. 1998 Oct;89(10):379-84.

Abstract

A case of calcinosis cutis, appeared since childhood in a woman 73-years-old, affected by diabetes mellitus with complications, is described. This uncommon disorder is discussed on the basis of data from recent literature. Calcinosis cutis is a condition characterized by the deposition of crystals of calcium phosphate (hydroxyapatite) in the skin. Calcinosis cutis may be idiopathic or secondary. The idiopathic calcinosis cutis is uncommon, may be solitary or multiple, sporadic or associated with Down syndrome (MICC or "milialike idiopathic calcinosis cutis") and appears more often in childhood or adolescence. Secondary calcinosis cutis may appear in the course of juvenile dermatomyositis or in the form of systemic scleroderma named CREST syndrome (calcinosis cutis, Raynaud's phenomenon, esophageal dysfunction, sclerodactyly and telangectasia). Calcinosis cutis may also be seen later in the course of renal failure, associated with hyperphosphatemia and secondary hyperparathyroidism. In this case report, calcinosis cutis appeared early in life and the laboratory data showed normal erythrocyte sedimentation rate and leukocyte count, negative LE test and absence of rheumatoid factor and non-organ-specific auto-antibodies, and multiple localizations. On these grounds, the diagnosis of idiopathic multiple calcinosis cutis was made. This is a rare and benign syndrome, which does not cause any late complication and whose prognosis is therefore favourable.

摘要

本文描述了一例皮肤钙质沉着症病例,该病例出现在一名73岁患有糖尿病并伴有并发症的女性身上,自童年起发病。本文依据近期文献资料对这种罕见病症进行了讨论。皮肤钙质沉着症是一种以皮肤中磷酸钙(羟基磷灰石)晶体沉积为特征的病症。皮肤钙质沉着症可分为特发性或继发性。特发性皮肤钙质沉着症较为罕见,可为单发或多发,散发性或与唐氏综合征相关(MICC或“类家族性特发性皮肤钙质沉着症”),且更常出现在儿童期或青春期。继发性皮肤钙质沉着症可能出现在青少年皮肌炎病程中,或以系统性硬化症的CREST综合征形式出现(皮肤钙质沉着症、雷诺现象、食管功能障碍、指端硬化和毛细血管扩张)。皮肤钙质沉着症也可能在肾衰竭病程后期出现,与高磷血症和继发性甲状旁腺功能亢进有关。在本病例报告中,皮肤钙质沉着症在早年出现,实验室检查数据显示红细胞沉降率和白细胞计数正常,狼疮细胞试验阴性,类风湿因子及非器官特异性自身抗体均不存在,且有多处病变。基于这些情况,诊断为特发性多发性皮肤钙质沉着症。这是一种罕见的良性综合征,不会引起任何晚期并发症,因此预后良好。

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