Arseculeratne G, Evans A T, Morley S M
Department of Dermatology, Ninewells Hospital and Medical School, Dundee, Scotland, UK.
J Eur Acad Dermatol Venereol. 2006 May;20(5):493-502. doi: 10.1111/j.1468-3083.2006.01506.x.
'Calciphylaxis', a calcification syndrome associated with ischaemic cutaneous necrosis, is acquired naturally in humans in disease states. It is a life and limb-threatening complication, usually observed in patients with renal disease and secondary hyperparathyroidism, but known to occur in the absence of renal or parathyroid disease. The reported mortality rate, which ranges from 60-80%, relates to wound infection, sepsis and organ failure. It is a small-vessel vasculopathy, which is estimated to occur in about 4% of haemodialysis patients. Clinically, violaceous, reticulate areas of cutaneous necrosis and eschar may be evident, particularly in the extremities. In addition to the clinical picture, a raised calcium phosphorous product, an elevated parathyroid hormone level, radiographic evidence of vessel and soft-tissue calcification and the finding of mural calcification affecting small arteries and arterioles on histopathology help to confirm the diagnosis of this entity which generally has a poor prognosis. A high index of suspicion and an active multidisciplinary management approach, with rigorous attention to wound care and prevention of sepsis, are vital in the management of these patients. In this overview, we discuss the pathophysiology, clinical features and associations, risk factors, diagnosis and management issues relating to calciphylaxis.
“钙化防御”是一种与缺血性皮肤坏死相关的钙化综合征，在人类疾病状态下自然发生。它是一种危及生命和肢体的并发症，通常见于肾病和继发性甲状旁腺功能亢进患者，但也已知在无肾病或甲状旁腺疾病的情况下发生。报道的死亡率在60%至80%之间，与伤口感染、败血症和器官衰竭有关。它是一种小血管血管病变，估计约4%的血液透析患者会发生。临床上，皮肤坏死和焦痂的紫红色网状区域可能很明显，尤其是在四肢。除临床表现外，钙磷乘积升高、甲状旁腺激素水平升高、血管和软组织钙化的影像学证据以及组织病理学上发现影响小动脉和小动脉的壁钙化有助于确诊这一通常预后不良的疾病。高度的怀疑指数和积极的多学科管理方法，严格关注伤口护理和预防败血症，对这些患者的管理至关重要。在本综述中，我们讨论了与钙化防御相关的病理生理学、临床特征和关联、危险因素、诊断和管理问题。
