你绝没见过的罕见病例——钙化防御
A图：左侧下肢难愈性溃疡及其坏死边缘
B图：X线显示左下肢弥漫性网状钙化动脉影及栓塞
C图：苏木素-伊红染色×200，溃疡部皮肤样本组织学分析显示血管壁钙化和血管内血栓形成
D图：von Kossa染色，动脉中的钙沉积物，被染成黑色颗粒的钙沉积物
▶A 59-year-old man with end-stage renal disease and haemodialysis since 2008 was admitted with painful, non-healing ulcerations on his legs. The lesions developed without known trauma 2 months before admission (figure). Pedal pulses were palpable on both sides and there were no signs of chronic venous insufficiency.
▶Radiography of the soft tissue revealed diffuse calcified arterioles in a mesh-like pattern (figure).
▶Histological examination of skin biopsy showed sclerosis and thrombosis of blood vessels (figure) and von Kossa stains were positive for calcium deposits (figure), confirming the diagnosis of calciphylaxis.
▶Calciphylaxis is a syndrome of systemic medial calcification of the arteries leading to tissue necrosis. Skin biopsy and radiographic features are helpful in the diagnosis; but negative results do not necessarily exclude the diagnosis.
▶Although the pathogenesis remains unclear, several comorbid conditions are known to increase its development. Associated risk factors in our patient were: end-stage renal disease, secondary hyperparathyroidism, and oral anticoagulation with phenprocoumon.
▶一59岁终末期肾病（从2008年起接受血液透析治疗）男性患者因下肢疼痛及难愈性溃疡来就诊。
▶患者于入院2个月前在无外伤的情况下发生下肢溃疡（附图）。患者双侧足动脉搏动明显，无慢性静脉功能不全。
▶X线示软组织弥漫性网状钙化动脉影（附图）。
▶皮肤样本组织学分析显示血管壁钙化和血管内血栓形成（附图），von Kossa染色中钙沉积物呈阳性（附图）， 因此确诊为钙化防御。
▶Recent reports indicate that low parathyroid hormone levels in association with adynamic bone diseases can also be associated with calciphylaxis.
▶Unfortunately, there is still no standardised treatment for calciphylaxis. Based on previous reports, phenprocoumon was withdrawn in our patient and he was treated with cinacalcet, daily haemodialysis, sodium thiosulfate, and bisphosphonate. After 4 weeks, skin condition improved and the patient was discharged home.
▶钙化防御是一种导致组织缺血坏死的系统性动脉中层钙化综合征。皮肤活检和X线检查具有诊断意义， 但两者的阴性结果不能排除该病的诊断。
▶目前钙化防御的发病机制尚不明确，但已知其他合并因素可促进疾病发生。
▶本例中的相关危险因素包括终末期肾病、继发性甲状旁腺功能亢进及口服苯丙香豆素类抗凝血药。最近有报道指出甲状旁腺激素水平较低合并动力缺失性骨病也与钙化防御有关。
▶遗憾的是目前尚无针对钙化防御的标准化治疗方案。基于既往的报道，停用苯丙香豆素类抗凝血药，予西那卡塞、硫代硫酸钠、双膦酸盐并每日透析。4周后，皮损改善，患者出院。
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翻译：郝跃文