Strupp Kim M, Zieg Jennifer A, Johnson Brian, Szolnoki Judit M
From the Department of Anesthesiology, Children's Hospital Colorado, University of Colorado, Aurora, Colorado.
A A Case Rep. 2017 Aug 1;9(3):73-76. doi: 10.1213/XAA.0000000000000530.
Epidermolysis bullosa (EB) encompasses a wide spectrum of rare genetic disorders in which an abnormality in collagen leads to loss or absence of normal intracellular bridges. Friction or shear forces on the skin and mucosa result in blister, bullae, and scar formation. We present our experience in the management of a patient with EB who required multiple procedures for squamous cell carcinoma of the left arm, including forequarter amputation. We describe the anesthetic challenges in caring for a patient with EB undergoing major orthopedic surgery.
大疱性表皮松解症（EB）涵盖了一系列罕见的遗传性疾病，其中胶原蛋白异常会导致正常细胞内桥粒缺失或缺乏。皮肤和黏膜受到摩擦或剪切力会导致水疱、大疱和瘢痕形成。我们介绍了我们对一名患有EB的患者的治疗经验，该患者因左臂鳞状细胞癌需要进行多次手术，包括上肢截肢术。我们描述了护理一名接受大型骨科手术的EB患者时所面临的麻醉挑战。
