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

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• 作者：Christina L. Boull ; MD¹ ; ^{oehr0005@umn.edu" class="auth_mail" title="E-mail the corresponding author} ; Sara A. Hylwa ; MD¹ ; Dusan Sajic ; MD² ; John E. Wagner ; MD³ ; Jakub Tolar ; MD ; PhD³ ; Kristen P. Hook ; MD¹
• 关键词：toxic epidermal necrolysis ; epidermolysis bullosa ; bone marrow transplantation ; mesenchymal stem cells
• 刊名：The Journal of Pediatrics
• 出版年：2016
• 出版时间：June 2016
• 年：2016
• 卷：173
• 期：Complete
• 页码：242-244
• 全文大小：420 K

文摘

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.