Plasmapheresis for refractory toxic epidermal necrolysis unresponsive to conventional therapy: a case report and literature review

血浆置换治疗对常规疗法无效的难治性中毒性表皮坏死松解症:病例报告及文献综述

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Abstract

Toxic epidermal necrolysis (TEN) is a rare and life-threatening severe cutaneous adverse reaction. The conventional treatment approach includes immunomodulatory therapies, such as systemic corticosteroids, cyclosporine, intravenous immunoglobulin (IVIG), and tumor necrosis factor-alpha (TNF-α) inhibitors. Plasmapheresis, as a potential treatment for TEN, is rarely used in patients with refractory TEN. We report a successful case of plasmapheresis treatment in a patient with refractory TEN who did not respond to conventional treatment, and we provide a literature review. A 65-year-old female presented with diffuse erythematous papules covering her entire body, along with multiple blisters and bullae, and partial detachment of the epidermis and mucosa. The area of epidermal exfoliation exceeded 30% of the total body surface area, and Nikolsky's sign was positive. Despite a week of methylprednisolone treatment, numerous blisters and bullae developed, and the area of epidermal exfoliation expanded to 62%. IVIG and TNF-α inhibitors were subsequently added, but the disease remained uncontrolled. Plasmapheresis treatment was initiated. Epithelial regeneration was observed after three days of plasmapheresis. After plasmapheresis was given 5 times, the patient finally recovered. This case highlights the significance of plasmapheresis in the treatment of refractory TEN, particularly when conventional therapies are ineffective. More studies are needed in the future to confirm the efficacy of plasmapheresis treatment.

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