Wollina U
Klinik für Dermatologie und Allergologie, Krankenhaus Dresden-Friedrichstadt, Städtisches Klinikum, Akademisches Lehrkrankenhaus der Technischen Universität Dresden, Friedrichstr. 41, 01067, Dresden.
Hautarzt. 2010 Dec;61(12):1063-71; quiz 1072. doi: 10.1007/s00105-010-2026-4.
Calciphylaxis represents a dermatological emergency with a mortality of up to 80%. The disease is characterized by a triad of arteriolar medial calcification, thrombotic cutaneous ischemia and necrotic ulcerations. Recently several mechanisms of vascular calcification have been identified. This may led to preventive measures in the future. Early diagnosis is important to avoid complications such as sepsis. The dermatologist plays an important role in early diagnosis based on the recognition of clinical presentation and histopathology. Patients with end-stage renal disease are most commonly affected by calciphylaxis. The most frequent non-uremic predisposing conditions are primary hyperparathyroidism, malignancies, alcohol-induced liver disease, and autoimmune connective tissue diseases. Medical treatment aims to normalize mineral metabolism to reduce the serum concentration of sodium phosphate and thus to prevent precipitation and calcification. Newer compounds are bisphosphonates, non-sodium/non-aluminium phosphate binders, cinacalcet, paricalcitrol, and sodium thiosulfate. Among the surgical procedures parathyroidectomy did not result in a significant survival benefit. An aggressive surgical debridement of necrotic ulcerations, on the other hand, improved survival. Early diagnosis and a multidisciplinary treatment approach including re-vascularization by the vascular surgeon, repeated surgical debridement and split skin transplantation support wound healing and insure limb conservation.
钙过敏症是一种皮肤科急症,死亡率高达80%。该病的特征为小动脉中层钙化、血栓性皮肤缺血和坏死性溃疡三联征。最近,已确定了几种血管钙化机制。这可能会在未来带来预防措施。早期诊断对于避免诸如败血症等并发症很重要。皮肤科医生基于对临床表现和组织病理学的认识在早期诊断中发挥重要作用。终末期肾病患者最常受到钙过敏症的影响。最常见的非尿毒症诱发因素是原发性甲状旁腺功能亢进、恶性肿瘤、酒精性肝病和自身免疫性结缔组织病。医学治疗旨在使矿物质代谢正常化,以降低血清磷酸钠浓度,从而防止沉淀和钙化。较新的化合物有双膦酸盐、非钠/非铝磷酸盐结合剂、西那卡塞、帕立骨化醇和硫代硫酸钠。在外科手术中,甲状旁腺切除术并未带来显著的生存获益。另一方面,积极的坏死性溃疡手术清创可提高生存率。早期诊断以及包括血管外科医生进行血管重建、重复手术清创和分层皮肤移植在内的多学科治疗方法有助于伤口愈合并确保保肢。