Dermatology, Venereology & Leprosy, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India.
Dermatology, Venereology & Leprosy, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India
BMJ Case Rep. 2023 Aug 9;16(8):e253507. doi: 10.1136/bcr-2022-253507.
We report a case of refractory paediatric pemphigus vulgaris with sepsis, treated successfully with intravenous immunoglobulin (IVIG) and amniotic membrane dressing. The patient was initially started on oral prednisolone (1 mg/kg/day) and dapsone 50 mg once daily. Azathioprine 50 mg orally was then used in place of dapsone due to rapid disease progression with extensive skin involvement. However, the patient developed sepsis and azathioprine had to be discontinued. Because of rapidly progressive disease and sepsis, the patient was put on IVIG at a dose of 2 g/kg in divided doses over 3 days along with amniotic membrane dressing. There was marked improvement after 2 weeks of follow-up.
我们报告了一例难治性儿童寻常型天疱疮合并脓毒症的病例，该病例成功地接受了静脉注射免疫球蛋白（IVIG）和羊膜敷料治疗。患者最初接受口服泼尼松龙（1mg/kg/天）和每日一次的氨苯砜 50mg 治疗。由于疾病迅速进展，皮肤广泛受累，随后用巯嘌呤 50mg 替代氨苯砜。然而，患者发生了脓毒症，必须停止使用巯嘌呤。由于疾病迅速进展和脓毒症，患者接受了 2g/kg 的 IVIG 剂量，分 3 天给予，同时进行羊膜敷料治疗。在随访 2 周后，病情明显改善。
