Toxic Epidermal Necrolysis in Recessive Dystrophic Epidermolysis Bullosa following Bone Marrow Transplantation. 2016

Christina L Boull, and Sara A Hylwa, and Dusan Sajic, and John E Wagner, and Jakub Tolar, and Kristen P Hook
Department of Dermatology, University of Minnesota, Minneapolis, MN. Electronic address: oehr0005@umn.edu.

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.

UI MeSH Term Description Entries
D007166 Immunosuppressive Agents Agents that suppress immune function by one of several mechanisms of action. Classical cytotoxic immunosuppressants act by inhibiting DNA synthesis. Others may act through activation of T-CELLS or by inhibiting the activation of HELPER CELLS. While immunosuppression has been brought about in the past primarily to prevent rejection of transplanted organs, new applications involving mediation of the effects of INTERLEUKINS and other CYTOKINES are emerging. Immunosuppressant,Immunosuppressive Agent,Immunosuppressants,Agent, Immunosuppressive,Agents, Immunosuppressive
D008297 Male Males
D002675 Child, Preschool A child between the ages of 2 and 5. Children, Preschool,Preschool Child,Preschool Children
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D013262 Stevens-Johnson Syndrome Rare cutaneous eruption characterized by extensive KERATINOCYTE apoptosis resulting in skin detachment with mucosal involvement. It is often provoked by the use of drugs (e.g., antibiotics and anticonvulsants) or associated with PNEUMONIA, MYCOPLASMA. It is considered a continuum of Toxic Epidermal Necrolysis. Drug-Induced Stevens Johnson Syndrome,Drug-Induced Stevens-Johnson Syndrome,Epidermal Necrolysis, Toxic,Lyell's Syndrome,Mycoplasma-Induced Stevens Johnson Syndrome,Mycoplasma-Induced Stevens-Johnson Syndrome,Nonstaphylococcal Scalded Skin Syndrome,Scalded Skin Syndrome, Nonstaphylococcal,Stevens Johnson Syndrome Toxic Epidermal Necrolysis,Stevens Johnson Syndrome Toxic Epidermal Necrolysis Spectrum,Stevens-Johnson Syndrome Toxic Epidermal Necrolysis,Stevens-Johnson Syndrome Toxic Epidermal Necrolysis Spectrum,Toxic Epidermal Necrolysis,Toxic Epidermal Necrolysis Stevens Johnson Syndrome,Toxic Epidermal Necrolysis Stevens Johnson Syndrome Spectrum,Toxic Epidermal Necrolysis Stevens-Johnson Syndrome,Toxic Epidermal Necrolysis Stevens-Johnson Syndrome Spectrum,Drug Induced Stevens Johnson Syndrome,Drug-Induced Stevens-Johnson Syndromes,Epidermal Necrolyses, Toxic,Lyell Syndrome,Lyell's Syndromes,Mycoplasma Induced Stevens Johnson Syndrome,Necrolyses, Toxic Epidermal,Necrolysis, Toxic Epidermal,Stevens Johnson Syndrome,Stevens-Johnson Syndrome, Drug-Induced,Stevens-Johnson Syndrome, Mycoplasma-Induced,Stevens-Johnson Syndromes, Drug-Induced,Syndrome, Lyell's,Syndrome, Mycoplasma-Induced Stevens-Johnson,Syndromes, Lyell's,Toxic Epidermal Necrolyses
D016026 Bone Marrow Transplantation The transference of BONE MARROW from one human or animal to another for a variety of purposes including HEMATOPOIETIC STEM CELL TRANSPLANTATION or MESENCHYMAL STEM CELL TRANSPLANTATION. Bone Marrow Cell Transplantation,Grafting, Bone Marrow,Transplantation, Bone Marrow,Transplantation, Bone Marrow Cell,Bone Marrow Grafting
D016108 Epidermolysis Bullosa Dystrophica Form of epidermolysis bullosa characterized by atrophy of blistered areas, severe scarring, and nail changes. It is most often present at birth or in early infancy and occurs in both autosomal dominant and recessive forms. All forms of dystrophic epidermolysis bullosa result from mutations in COLLAGEN TYPE VII, a major component fibrils of BASEMENT MEMBRANE and EPIDERMIS. Cockayne-Touraine Disease,Epidermolysis Bullosa, Dystrophic,Hallopeau-Siemens Disease,Cockayne-Touraine Type Epidermolysis Bullosa,Dystrophic Epidermolysis Bullosa,Dystrophic Epidermolysis Bullosa, Autosomal Recessive,Epidermolysis Bullosa Dystrophica, Autosomal Recessive,Epidermolysis Bullosa Dystrophica, Cockayne-Touraine Type,Epidermolysis Bullosa Dystrophica, Dominant,Epidermolysis Bullosa Dystrophica, Hallopeau-Siemens Type,Epidermolysis Bullosa Dystrophica, Recessive,Bullosa Dystrophica, Epidermolysis,Bullosa Dystrophicas, Epidermolysis,Bullosa, Dystrophic Epidermolysis,Bullosas, Dystrophic Epidermolysis,Cockayne Touraine Disease,Cockayne Touraine Type Epidermolysis Bullosa,Dystrophic Epidermolysis Bullosas,Dystrophica, Epidermolysis Bullosa,Dystrophicas, Epidermolysis Bullosa,Epidermolysis Bullosa Dystrophica, Cockayne Touraine Type,Epidermolysis Bullosa Dystrophica, Hallopeau Siemens Type,Epidermolysis Bullosa Dystrophicas,Epidermolysis Bullosas, Dystrophic,Hallopeau Siemens Disease
D016572 Cyclosporine A cyclic undecapeptide from an extract of soil fungi. It is a powerful immunosupressant with a specific action on T-lymphocytes. It is used for the prophylaxis of graft rejection in organ and tissue transplantation. (From Martindale, The Extra Pharmacopoeia, 30th ed). Cyclosporin A,Ciclosporin,CsA-Neoral,CyA-NOF,Cyclosporin,Cyclosporine A,Neoral,OL 27-400,Sandimmun,Sandimmun Neoral,Sandimmune,CsA Neoral,CsANeoral,CyA NOF,OL 27 400,OL 27400
D045164 Mesenchymal Stem Cell Transplantation Transfer of MESENCHYMAL STEM CELLS between individuals within the same species (TRANSPLANTATION, HOMOLOGOUS) or transfer within the same individual (TRANSPLANTATION, AUTOLOGOUS). Stem Cell Transplantation, Mesenchymal,Transplantation, Mesenchymal Stem Cell

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