Chen Tiffany Y, Lehman Julia S, Gibson Lawrence E, Lohse Christine M, El-Azhary Rokea A
*Medical Student, Mayo Medical School, Mayo Clinic, Rochester, MN; †Associate Professor, Department of Dermatology, Mayo Clinic, Rochester, MN; ‡Professor, Departments of Dermatology and Laboratory Medicine and Pathology, Mayo Clinic, Rochester, MN; §Biostatistician, Department of Health Sciences Research, Mayo Clinic, Rochester, MN; and ‖Professor, Department of Dermatology, Mayo Clinic, Rochester, MN.
Am J Dermatopathol. 2017 Nov;39(11):795-802. doi: 10.1097/DAD.0000000000000824.
Calciphylaxis is a rare, painful, and life-threatening condition with a high mortality rate. Although the etiology of calciphylaxis is not well understood, it has been proposed that calcium deposition within and around subcutaneous vessels restricts blood flow chronically, thereby predisposing the patient to acute pannicular and dermal thrombosis. Given increasing recognition of the role of hypercoagulability in calciphylaxis, this retrospective cohort study sought to evaluate the presence of thromboses and dermal angioplasia in calciphylaxis. Moreover, we aimed to validate previous observations about the histopathology of calciphylaxis compared with skin biopsies from patients with end-stage renal disease but without calciphylaxis. After a meticulous clinical chart review, we assessed the corresponding skin biopsies for the presence of vessel calcification, thromboses, and dermal angioplasia in skin biopsies from patients with calciphylaxis (n = 57) and compared with those from patients with end-stage renal disease but without calciphylaxis (n = 26). Histopathologic findings were correlated with clinical features such as chronic kidney disease, diabetes, or associated malignancy. Our results validated a prior observation that calciphylaxis was significantly more likely to show calcification of dermal vessels and diffuse dermal thrombi. This study reports the frequent finding of dermal angioplasia, a potential marker of chronic low-grade ischemia, as another frequent microscopic finding in calciphylaxis. Among cases of calciphylaxis, histopathologic changes in patients with chronic kidney disease were indistinguishable from those in patients without chronic kidney disease, thereby implying a final common pathogenic pathway in both uremic and nonuremic calciphylaxis. In future, larger, prospective studies may be useful in validating these findings.
钙过敏症是一种罕见、痛苦且危及生命的疾病，死亡率很高。尽管钙过敏症的病因尚未完全明确，但有人提出皮下血管内及周围的钙沉积会长期限制血液流动，从而使患者易患急性脂肪小叶和皮肤血栓形成。鉴于对高凝状态在钙过敏症中作用的认识不断增加，这项回顾性队列研究旨在评估钙过敏症中血栓形成和皮肤血管生成的情况。此外，我们旨在验证先前关于钙过敏症组织病理学的观察结果，并与终末期肾病但无钙过敏症患者的皮肤活检结果进行比较。在仔细查阅临床病历后，我们评估了钙过敏症患者（n = 57）皮肤活检中血管钙化、血栓形成和皮肤血管生成的情况，并与终末期肾病但无钙过敏症患者（n = 26）的活检结果进行比较。组织病理学发现与慢性肾病、糖尿病或相关恶性肿瘤等临床特征相关。我们的结果验证了先前的一项观察结果，即钙过敏症更有可能出现真皮血管钙化和弥漫性真皮血栓形成。本研究报告了皮肤血管生成这一慢性低度缺血的潜在标志物在钙过敏症中也是常见的微观发现。在钙过敏症病例中，慢性肾病患者的组织病理学变化与无慢性肾病患者的变化无法区分，这意味着尿毒症性和非尿毒症性钙过敏症存在共同的最终致病途径。未来，更大规模的前瞻性研究可能有助于验证这些发现。
