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[与肾功能不全相关的钙化性脂膜炎:一种组织钙化防御综合征]

[Calcifying panniculitis associated with renal insufficiency: a tissue calciphylaxis syndrome].

作者信息

Laurent R, Thiery F, Saint-Hillier Y, Blanc D, Agache P

机构信息

Département de Dermatologie, CHU Saint-Jacques, Besançon.

出版信息

Ann Dermatol Venereol. 1987;114(9):1073-81.

PMID:2963579
Abstract

Among the cutaneous manifestations of hyperparathyroidism, cases of panniculitis with calcification of the adipose tissue and necrosis of the skin have recently been reported, the mechanism incriminated being calciphylaxis, as defined by Selye on the basis of experiments. Experimental calciphylaxis consists of local or systemic calcium deposits followed by inflammatory necrosis or sclerosis. The deposits are induced by "provoking" or precipitating factors (metal salts, albumin, traumas) after a phase of sensitization (to parathyroid hormone, vitamins D2 or D3, dihydrotachysterol), provided a critical period is allowed between these two phases; the duration of that period depends on the experimental conditions. The case reported here concerns a 64-year old obese and diabetic woman who had presented with hard and tender nodosities and plaques in her abdominal and crural panniculi, ending in extensive and hyperalgesic necrosis (fig. 1 and 2). The panniculitis had occurred in a peculiar context: at the end of an episode of renal failure complicated with secondary hyperparathyroidism (serum PTH 12.9 mIU/ml; N = 1.5-4.4 mIU/ml) with moderate increase to 5,000 of the P x Ca product. Histological examination of a nodule of the thigh disclosed multiple foci of microcalcification (fig. 3, 4, 5) within the adipose lobules, in the interadipocyte spaces, in connective tissue septa and in the adventitia of small vessels (positive Von Kossa reaction). Electron microscopy showed dense calcium deposits between adipocytes, in subcutaneous septa (fig. 6, 8) and in more or less damaged vascular walls (fig. 9). Within the microfibrillar and granular fundamental substance, microcrystals looking like hydroxyapatite crystals (fig. 7) conglomerated into pincushion-like formations becoming increasingly denser and more compact.(ABSTRACT TRUNCATED AT 250 WORDS)

摘要

在甲状旁腺功能亢进的皮肤表现中,最近有脂肪组织钙化和皮肤坏死的脂膜炎病例报道,其发病机制被认为是钙质沉着症,这是塞利基于实验所定义的。实验性钙质沉着症包括局部或全身钙沉积,随后出现炎症性坏死或硬化。这些沉积是在致敏阶段(对甲状旁腺激素、维生素D2或D3、双氢速甾醇)之后,由“激发”或促发因素(金属盐、白蛋白、创伤)诱导产生的,前提是在这两个阶段之间有一个关键时期;该时期的持续时间取决于实验条件。本文报道的病例是一名64岁的肥胖糖尿病女性,其腹部和腿部脂膜出现坚硬且触痛的结节及斑块,最终发展为广泛且疼痛加剧的坏死(图1和图2)。脂膜炎发生在一个特殊背景下:在一次肾衰竭并发继发性甲状旁腺功能亢进(血清甲状旁腺激素12.9 mIU/ml;正常范围1.5 - 4.4 mIU/ml)且钙磷乘积中度升高至5000之后。对大腿结节的组织学检查显示,在脂肪小叶内、脂肪细胞间隙、结缔组织间隔和小血管外膜有多个微钙化灶(冯·科萨反应阳性)(图3、4、5)。电子显微镜显示脂肪细胞之间、皮下间隔(图6、8)以及或多或少受损的血管壁(图9)中有密集的钙沉积。在微纤维和颗粒状基质内,类似羟基磷灰石晶体的微晶(图7)聚集成针垫状结构,且越来越致密和紧实。(摘要截取自250词)

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