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静脉注射免疫球蛋白和羊膜敷料治疗难治性儿童寻常型天疱疮合并脓毒症。

Rescue treatment with intravenous immunoglobulin and amniotic membrane dressing in refractory paediatric pemphigus vulgaris with sepsis.

机构信息

Dermatology, Venereology & Leprosy, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India.

Dermatology, Venereology & Leprosy, All India Institute of Medical Sciences, Rishikesh, Uttarakhand, India

出版信息

BMJ Case Rep. 2023 Aug 9;16(8):e253507. doi: 10.1136/bcr-2022-253507.

Abstract

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.

摘要

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。

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本文引用的文献

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Use of amniotic membrane in dermatology.羊膜在皮肤病学中的应用。
Indian J Dermatol Venereol Leprol. 2010 Mar-Apr;76(2):196-7. doi: 10.4103/0378-6323.60565.
5
Treatment of juvenile pemphigus vulgaris with intravenous immunoglobulin therapy.静脉注射免疫球蛋白疗法治疗青少年寻常型天疱疮。
Pediatr Dermatol. 2009 Mar-Apr;26(2):197-202. doi: 10.1111/j.1525-1470.2008.00808.x. Epub 2009 Mar 11.

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