Sato N, Teramura T, Ishiyama T, Tagami H
Department of Dermatology, Akita Kumiai General Hospital, Japan.
J Dermatol. 2001 Jan;28(1):27-31. doi: 10.1111/j.1346-8138.2001.tb00082.x.
A 50-year-old Japanese female with chronic renal failure who had been on continuous ambulatory peritoneal dialysis developed fulminant systemic cutaneous necrosis that began as painful livedo reticularis-like skin lesions on her thighs. Because of disseminated vascular calcification within the muscular layer of her lower limbs, we eventually diagnosed her with calciphylaxis. The skin necrosis progressed rapidly, and she died of sepsis and pneumonia on the 53rd hospital day. In addition to her long-lasting severe hyperparathyroidism and extremely elevated serum phosphorus and calcium levels, mechanical, frictional stimulation inflicted on the local skin and administration of corticosteroids were suspected to have precipitated the calciphylaxis. Our lack of awareness of this disease in its early stages resulted in our missing the chance to do a parathyroidectomy that might have changed the course. It is important to know the clinical features of this rare disease in order to make a diagnosis as early as possible.
一名50岁的日本女性，患有慢性肾衰竭，一直在进行持续性非卧床腹膜透析，她出现了暴发性全身性皮肤坏死，最初表现为大腿上类似网状青斑的疼痛性皮肤病变。由于其下肢肌肉层内存在弥漫性血管钙化，我们最终诊断她患有钙化防御。皮肤坏死迅速进展，她在住院第53天死于败血症和肺炎。除了长期严重的甲状旁腺功能亢进以及血清磷和钙水平极度升高外，局部皮肤受到的机械性、摩擦性刺激以及使用皮质类固醇被怀疑促使了钙化防御的发生。我们在疾病早期对这种疾病缺乏认识，导致我们错失了进行甲状旁腺切除术的机会，而这可能会改变病程。了解这种罕见疾病的临床特征对于尽早做出诊断很重要。
