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Case Reports
. 2016 Jun:173:242-4.
doi: 10.1016/j.jpeds.2016.02.037. Epub 2016 Mar 11.

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

Christina L Boull  1 , Sara A Hylwa  2 , Dusan Sajic  3 , John E Wagner  4 , Jakub Tolar  4 , Kristen P Hook  2
Affiliations
Case Reports

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

Christina L Boull et al. J Pediatr. 2016 Jun.

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.

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Figures

Figure 1
Figure 1
Patient with full thickness epidermal necrolysis covering 90% of his body.
Figure 2
Figure 2
Biopsy showing full thickness epidermal necrosis with sparse inflammation.
Figure 3
Figure 3
Patient with diffuse firm, flesh colored, folliculocentric papules.
See this image and copyright information in PMC

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