Toxic epidermal necrolysis in recessive dystrophic epidermolysis bullosa following bone marrow transplantation

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4 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)242-244
Number of pages3
JournalJournal of Pediatrics
Volume173
DOIs
StatePublished - Jun 1 2016

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Epidermolysis Bullosa Dystrophica
Stevens-Johnson Syndrome
Bone Marrow Transplantation
Epidermolysis Bullosa
Re-Epithelialization
Biopsy
Skin
Clinical Laboratory Techniques
Graft vs Host Disease
Mesenchymal Stromal Cells
Cyclosporine
Transplantation
Bone Marrow
Transplants
Therapeutics

Keywords

  • bone marrow transplantation
  • epidermolysis bullosa
  • mesenchymal stem cells
  • toxic epidermal necrolysis

Cite this

@article{9674296715af406ead7c5ea3f88ee686,
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.",
keywords = "bone marrow transplantation, epidermolysis bullosa, mesenchymal stem cells, toxic epidermal necrolysis",
author = "Boull, {Christina L.} and Hylwa, {Sara A.} and Dusan Sajic and Wagner, {John E.} and Jakub Tolar and Hook, {Kristen P.}",
year = "2016",
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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.

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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