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

• Published in: The Journal of pediatrics Vol. 173; pp. 242 - 244
• Main Authors: Boull, Christina L., MD, Hylwa, Sara A., MD, Sajic, Dusan, MD, Wagner, John E., MD, Tolar, Jakub, MD, PhD, Hook, Kristen P., MD
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01.06.2016
• Subjects: bone marrow transplantation; Bone Marrow Transplantation - adverse effects; Child, Preschool; Cyclosporine - therapeutic use; epidermolysis bullosa; Epidermolysis Bullosa Dystrophica - therapy; Humans; Immunosuppressive Agents - therapeutic use; Male; Mesenchymal Stem Cell Transplantation; mesenchymal stem cells; Pediatrics; Stevens-Johnson Syndrome - etiology; Stevens-Johnson Syndrome - therapy; toxic epidermal necrolysis; Hematopoietic stem cell transplant; Mesenchymal stem cell; epidermolysis bullosa; mesenchymal stem cells; Intravenous immunoglobulin; IVIG; MSC; TEN; HSCT; bone marrow transplantation; toxic epidermal necrolysis
• ISSN: 0022-3476; 1097-6833
• DOI: 10.1016/j.jpeds.2016.02.037

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.