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骨髓移植后隐性营养不良型大疱性表皮松解症中的中毒性表皮坏死松解症

Toxic Epidermal Necrolysis in Recessive Dystrophic Epidermolysis Bullosa following Bone Marrow Transplantation.

作者信息

Boull Christina L, Hylwa Sara A, Sajic Dusan, Wagner John E, Tolar Jakub, Hook Kristen P

机构信息

Department of Dermatology, University of Minnesota, Minneapolis, MN.

Department of Dermatology, University of Minnesota, Minneapolis, MN.

出版信息

J Pediatr. 2016 Jun;173:242-4. doi: 10.1016/j.jpeds.2016.02.037. Epub 2016 Mar 11.

Abstract

A 3-year-old child with recessive dystrophic epidermolysis bullosa treated with bone marrow transplantation subsequently developed body-wide epidermal detachment distinct from his epidermolysis bullosa. Toxic epidermal necrolysis was diagnosed by examination and skin biopsy. Although graft-vs-host disease was considered, he had no features of this diagnosis by laboratory studies or skin biopsy, and he improved without addition of further immune suppressants. Throughout the episode, the patient was maintained on cyclosporine A, a component of his transplant regimen, and also a reported therapy for toxic epidermal necrolysis. He had full recovery. Re-epithelialization occurred in a unique folliculocentric pattern, which we postulate was related to the patient's mesenchymal stem cell infusion, received as an adjunct to his marrow transplantation.

摘要

一名患有隐性营养不良型大疱性表皮松解症的3岁儿童接受骨髓移植治疗后,出现了与他的大疱性表皮松解症不同的全身性表皮剥脱。通过检查和皮肤活检诊断为中毒性表皮坏死松解症。尽管考虑了移植物抗宿主病,但通过实验室检查或皮肤活检,他没有该诊断的特征,并且在未添加进一步免疫抑制剂的情况下病情好转。在整个病程中,患者一直使用环孢素A,这是他移植方案的一部分,也是一种报道的治疗中毒性表皮坏死松解症的疗法。他完全康复。上皮再形成以独特的毛囊中心模式发生,我们推测这与患者作为骨髓移植辅助治疗接受的间充质干细胞输注有关。

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