title: Severe Skin Complications After Small Bowel Transplantation: Graft-Versus-Host Disease, DRESS, Virus, or Drug Toxicity?
abstract: Severe skin problems are uncommon after small bowel transplantation. Differential diagnosis includes drug reactions, infections, graft-versus-host disease (GVHD), and mixed diseases. Early diagnosis and treatment are determinant for prognosis.
  
  
  
  We describe 6 patients with severe cutaneous complications after small bowel transplantation, the work-up, final diagnosis, and evolution. Two patients died from chronic GVHD or unrecognized drug rash with eosinophilia and systemic symptoms, the others recovered completely. In 2 patients, drugs and viruses could be implicated, and in 1 patient may have hidden or triggered chronic GVHD. Viruses (human herpesvirus 6, Epstein-Barr virus, and cytomegalovirus) were suspected to trigger drug rash with eosinophilia and systemic symptoms or GVHD. The 2 cases of acute GVHD were reversed completely by increased immunosuppression and anti-interleukin-2 receptor antibody.
  
  
  
  In these severe cases, diagnosis is urgent and should include a careful evaluation of drug history, clinical presentation, biological investigations, infections, and toxic screening. A skin biopsy and chimerism study should be performed whenever possible. An early treatment is key to a positive outcome.
authors.affiliation: 1 Department of Paediatric Hepatogastroenterology-Nutrition, Assistance Publique, Hôpitaux de Paris (AP-HP), Necker-Enfants Malades Hospital, Paris, France. 2 Department of Dermatology, AP-HP, Necker-Enfants Malades Hospital, Paris Descartes University, Sorbonne Paris Cité, Paris, France. 3 Department of Pathology, AP-HP, Necker-Enfants Malades Hospital, Paris, France. 4 Department of Paediatric Immuno-Haematology and Microbiology Laboratory, AP-HP, Necker-Enfants Malades Hospital, Paris Descartes University, Sorbonne Paris Cité, Paris, France. 5 Department of Paediatric Surgery, AP-HP, Necker-Enfants Malades Hospital, Paris, France.
authors.forename: Caroline
authors.initials: C
authors.lastname: Cruysmans
authors.forename: Marie-Gabrielle
authors.initials: MG
authors.lastname: Ferneiny
authors.forename: Sylvie
authors.initials: S
authors.lastname: Fraitag
authors.forename: Pierre
authors.initials: P
authors.lastname: Frange
authors.forename: Christophe
authors.initials: C
authors.lastname: Chardot
authors.forename: Christine
authors.initials: C
authors.lastname: Bodemer
authors.forename: Olivier
authors.initials: O
authors.lastname: Goulet
authors.forename: Florence
authors.initials: F
authors.lastname: Lacaille
mesh_terms: D002648:Child; D002675:Child, Preschool; D063926:Drug Hypersensitivity Syndrome / Q000209:etiology*; D005260:Female; D006086:Graft vs Host Disease / Q000209:etiology*; D006801:Humans; D007421:Intestine, Small / Q000637:transplantation*; D008297:Male; D011183:Postoperative Complications / Q000209:etiology*; D012871:Skin Diseases / Q000209:etiology*; D014777:Virus Diseases / Q000209:etiology*