Byrd Victoria, Nemeth Attila
Pediatrics, Case Western Reserve University School of Medicine, Cleveland, USA.
Internal Medicine, Louis Stokes Cleveland VA Medical Center/Case Western Reserve University School of Medicine, Cleveland, USA.
Cureus. 2018 Nov 19;10(11):e3608. doi: 10.7759/cureus.3608.
Epidermolysis bullosa acquisita is a rare autoimmune bullous disease involving the skin and mucosa, most commonly treated with systemic corticosteroids. This case illustrates the importance of counseling patients on medication side effects and ensuring close physician follow-up during an extended course of steroids. A 46-year-old man presented to the emergency department with weakness, fatigue, dizziness and polyuria in the setting of eight weeks of prednisone therapy for a flare-up of his bullous disease. Labs were significant for a blood glucose of 786 mg/dL, negative urine ketones, a normal anion gap, and an acute kidney injury. Blood glucose improved to 413 mg/dL after initial treatment with fluid and insulin. The patient was admitted and acute kidney injury resolved. He remained hyperglycemic despite his adjusted prednisone taper and corrective scale insulin, so basal and scheduled, pre-prandial insulins were added. After discharge, he was bridged to steroid-sparing therapy (rituximab). Physicians should counsel patients with epidermolysis bullosa acquisita about the risks of steroid-induced diabetes mellitus and its associated complications including hyperglycemic hyperosmolar state and diabetic ketoacidosis. Primary care physicians should screen for hyperglycemia during therapy and consider alternative treatments when necessary.
获得性大疱性表皮松解症是一种罕见的自身免疫性大疱性疾病,累及皮肤和黏膜,最常用全身糖皮质激素进行治疗。该病例说明了就药物副作用向患者提供咨询以及在长期使用糖皮质激素治疗过程中确保医生密切随访的重要性。一名46岁男性因大疱性疾病发作接受泼尼松治疗8周后,出现乏力、疲劳、头晕和多尿,前往急诊科就诊。实验室检查结果显示血糖为786 mg/dL、尿酮体阴性、阴离子间隙正常以及急性肾损伤。经补液和胰岛素初始治疗后,血糖降至413 mg/dL。患者入院,急性肾损伤得以缓解。尽管调整了泼尼松减量方案并使用了校正剂量的胰岛素,但患者仍存在高血糖,因此加用了基础胰岛素和餐前定时胰岛素。出院后,患者过渡到使用免疫抑制剂(利妥昔单抗)治疗。医生应向获得性大疱性表皮松解症患者告知糖皮质激素诱发糖尿病及其相关并发症(包括高血糖高渗状态和糖尿病酮症酸中毒)的风险。初级保健医生应在治疗期间筛查高血糖,并在必要时考虑采用替代治疗方法。