TY - JOUR
T1 - Toxic epidermal necrolysis in recessive dystrophic epidermolysis bullosa following bone marrow transplantation
AU - Boull, Christina L.
AU - Hylwa, Sara A.
AU - Sajic, Dusan
AU - Wagner, John E.
AU - Tolar, Jakub
AU - Hook, Kristen P.
N1 - Publisher Copyright:
© 2016 Elsevier Inc. All rights reserved.
PY - 2016/6/1
Y1 - 2016/6/1
N2 - 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.
AB - 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.
KW - bone marrow transplantation
KW - epidermolysis bullosa
KW - mesenchymal stem cells
KW - toxic epidermal necrolysis
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U2 - 10.1016/j.jpeds.2016.02.037
DO - 10.1016/j.jpeds.2016.02.037
M3 - Article
C2 - 26976809
AN - SCOPUS:84960423040
SN - 0022-3476
VL - 173
SP - 242
EP - 244
JO - Journal of Pediatrics
JF - Journal of Pediatrics
ER -