| Literature DB >> 26976809 |
Christina L Boull1, Sara A Hylwa2, Dusan Sajic3, John E Wagner4, Jakub Tolar4, Kristen P Hook2.
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.Entities:
Keywords: bone marrow transplantation; epidermolysis bullosa; mesenchymal stem cells; toxic epidermal necrolysis
Mesh:
Substances:
Year: 2016 PMID: 26976809 PMCID: PMC5322426 DOI: 10.1016/j.jpeds.2016.02.037
Source DB: PubMed Journal: J Pediatr ISSN: 0022-3476 Impact factor: 4.406