title: Toxic Epidermal Necrolysis in Recessive Dystrophic Epidermolysis Bullosa following Bone Marrow Transplantation.
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.
authors.affiliation: Department of Dermatology, University of Minnesota, Minneapolis, MN. Electronic address: oehr0005@umn.edu.
authors.forename: Christina L
authors.initials: CL
authors.lastname: Boull
authors.affiliation: Department of Dermatology, University of Minnesota, Minneapolis, MN.
authors.forename: Sara A
authors.initials: SA
authors.lastname: Hylwa
authors.affiliation: Department of Dermatology, University of Toronto, Toronto, Ontario, Canada.
authors.forename: Dusan
authors.initials: D
authors.lastname: Sajic
authors.affiliation: Division of Blood and Marrow Transplantation, University of Minnesota, Minneapolis, MN.
authors.forename: John E
authors.initials: JE
authors.lastname: Wagner
authors.affiliation: Division of Blood and Marrow Transplantation, University of Minnesota, Minneapolis, MN.
authors.forename: Jakub
authors.initials: J
authors.lastname: Tolar
authors.affiliation: Department of Dermatology, University of Minnesota, Minneapolis, MN.
authors.forename: Kristen P
authors.initials: KP
authors.lastname: Hook
mesh_terms: D016026:Bone Marrow Transplantation / Q000009:adverse effects*; D002675:Child, Preschool; D016572:Cyclosporine / Q000627:therapeutic use; D016108:Epidermolysis Bullosa Dystrophica / Q000628:therapy*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D045164:Mesenchymal Stem Cell Transplantation; D013262:Stevens-Johnson Syndrome / Q000209:etiology* / Q000628:therapy
chemical_list: D007166:Immunosuppressive Agents; D016572:Cyclosporine
keywords: bone marrow transplantation; epidermolysis bullosa; mesenchymal stem cells; toxic epidermal necrolysis