Sermijn E, Strobbe T, Vandekerckhove L, Libbrecht L, Colle I, Schoonjans R, Vogelaers D
Dienst Algemene Inwendige Ziekten, Infectieziekten & Psychosomatiek, UZ Gent, Belgium.
Acta Clin Belg. 2013 Mar-Apr;68(2):116-9. doi: 10.2143/ACB.2863.
Calciphylaxis, or calcific uremic arteriolopathy (CUA) is a rare but well described entity in patients with endstage renal disease (ESRD) and/or hyperparathyroidism. CUA is characterized by systemic acute calcification of the small and intermediate dermal vasculature that can lead to epidermal ischemia, ulceration, and necrosis. Cutaneous lesions of calciphylaxis characteristically begin as tender, violaceous, livedoid discolorations. The mechanisms of disease remain poorly understood although abnormal bone and mineral metabolism and hyperparathyroidism can contribute to CUA. Therapeutic strategies are of unproven benefit and mortality remains high. Calciphylaxis has also been extremely rarely reported in patients without ESRD and/or hyperparathyroidism. We report an unusual case of calciphylaxis in a patient with alcoholic liver cirrhosis and normal renal function, without any alteration in the phosphocalcic and parathyroid hormone (PTH) metabolisms.
钙化防御，即钙化性尿毒症小动脉病（CUA），在终末期肾病（ESRD）和/或甲状旁腺功能亢进患者中是一种罕见但已得到充分描述的病症。CUA的特征是皮肤中小动脉和中等大小动脉的系统性急性钙化，可导致表皮缺血、溃疡和坏死。钙化防御的皮肤病变特征性地始于压痛性、紫罗兰色、类紫癜样变色。尽管异常的骨和矿物质代谢以及甲状旁腺功能亢进可导致CUA，但该疾病的发病机制仍知之甚少。治疗策略的益处尚未得到证实，死亡率仍然很高。在没有ESRD和/或甲状旁腺功能亢进的患者中，钙化防御也极为罕见。我们报告了一例酒精性肝硬化且肾功能正常的患者发生钙化防御的不寻常病例，其钙磷和甲状旁腺激素（PTH）代谢没有任何改变。
