Nikko A P, Dunningan M, Cockerell C J
Division of Dermatopathology, University of Texas Southwestern Medical Center, Dallas, USA.
Am J Dermatopathol. 1996 Aug;18(4):396-9. doi: 10.1097/00000372-199608000-00011.
Calciphylaxis is a rare condition of widespread calcification of tissues and blood vessels with accompanying vascular thrombosis and ischemic necrosis. Most cases develop in association with hyperparathyroidism in patients with chronic renal failure. Pseudoxanthoma elasticum (PXE) is a hereditary condition of abnormal elastic tissue structure that leads to widespread abnormalities of the skin, retina, and visceral organs. Histologic changes of PXE have been observed as coincidental findings in several conditions such as following trauma to the skin manifest as isolated plaques often in scars. We observed histologic findings of PXE in a patient with chronic renal failure who developed fatal calciphylaxis. Complete evaluation failed to reveal evidence of systemic findings of PXE. Histologic changes of PXE may be seen in patients with calciphylaxis as a coincidental finding. Rapidly developing soft tissue calcification may lead to the expression of the characteristic histopathologic findings of PXE without evidence of classic clinical manifestations of PXE. Calciphylaxis should be added to the list of disorders that may lead to microscopic PXE-like changes.
钙过敏是一种罕见的疾病，表现为组织和血管广泛钙化，并伴有血管血栓形成和缺血性坏死。大多数病例发生于慢性肾衰竭患者并发甲状旁腺功能亢进时。弹性假黄瘤（PXE）是一种遗传性疾病，其弹性组织结构异常，导致皮肤、视网膜和内脏器官广泛出现异常。PXE的组织学改变在多种情况下作为偶然发现被观察到，比如皮肤创伤后表现为孤立斑块，常出现在瘢痕中。我们在一名发生致命性钙过敏的慢性肾衰竭患者中观察到了PXE的组织学表现。全面评估未发现系统性PXE表现的证据。PXE的组织学改变可能在钙过敏患者中作为偶然发现出现。快速发展的软组织钙化可能导致PXE特征性组织病理学表现的出现，而无PXE经典临床表现的证据。钙过敏应被列入可能导致微观PXE样改变的疾病清单中。
