Department of Dermatology, University Hospital "Dr. José Eleuterio González", Autonomous University of Nuevo León, Av. Madero y Gonzalitos S/N 64460 Col. Mitras Centro, Monterrey, Mexico.
Am J Clin Dermatol. 2018 Aug;19(4):599-608. doi: 10.1007/s40257-018-0361-x.
Calciphylaxis, also known as calcific uremic arteriolopathy and uremic small artery disease with medial wall calcification and intimal hyperplasia, is a multifactorial cutaneous vascular disease characterized by chronic, painful, non-healing wounds that occur frequently in patients with chronic kidney disease, predominantly in those with end-stage renal disease. The pathogenesis remains unclear, and the development of calciphylaxis lesions depends on medial calcification, intimal fibrosis of arterioles and thrombotic occlusion. Despite an increase in reports of calciphylaxis in the literature and clinical recognition of demographic characteristics and risk factors associated with calciphylaxis, it remains a poorly understood disease with high morbidity and mortality. In this review, we analyze and summarize the clinical manifestations, pathogenesis and pathophysiology, histopathology, differential diagnosis, diagnostic workup and treatment modalities for calciphylaxis. Because of the lack of consensus regarding the optimal approach to and treatment of this disorder, a high degree of clinical suspicion, early diagnosis, and multimodal and multidisciplinary treatment in collaboration with dermatology, nephrology, wound care, nutrition and pain management specialties may improve survival in patients with calciphylaxis.
钙化防御，亦称钙化尿毒症性小动脉病伴中膜钙化和内膜增生，是一种多因素的皮肤血管疾病，以慢性、疼痛、难以愈合的溃疡为特征，常发生于慢性肾脏病患者，尤其是终末期肾病患者。其发病机制尚不清楚，钙化防御病变的发展取决于中膜钙化、小动脉内膜纤维化和血栓闭塞。尽管文献中钙化防御的报道增加，且临床认识到与钙化防御相关的人口统计学特征和危险因素，但它仍然是一种了解甚少的疾病，具有高发病率和死亡率。在这篇综述中，我们分析和总结了钙化防御的临床表现、发病机制和病理生理学、组织病理学、鉴别诊断、诊断评估和治疗方法。由于缺乏针对这种疾病的最佳治疗方法的共识，因此高度的临床怀疑、早期诊断以及与皮肤科、肾病科、伤口护理、营养和疼痛管理专业合作的多模式和多学科治疗，可能会提高钙化防御患者的生存率。
