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: Christina L Boull, Sara A Hylwa, Dusan Sajic, John E Wagner, Jakub Tolar, Kristen P Hook
• Journal: The Journal of pediatrics (J Pediatr) Vol. 173 Pg. 242-4 (Jun 2016) ISSN: 1097-6833 [Electronic] United States
• PMID: 26976809 (Publication Type: Case Reports, Journal Article)
• Copyright: Copyright © 2016 Elsevier Inc. All rights reserved.

Chemical References

• Immunosuppressive Agents
• Cyclosporine

Topics

• Bone Marrow Transplantation (adverse effects)
• Child, Preschool
• Cyclosporine (therapeutic use)
• Epidermolysis Bullosa Dystrophica (therapy)
• Humans
• Immunosuppressive Agents (therapeutic use)
• Male
• Mesenchymal Stem Cell Transplantation
• Stevens-Johnson Syndrome (etiology, therapy)