Severe Skin Complications After Small Bowel Transplantation: Graft-Versus-Host Disease, DRESS, Virus, or Drug Toxicity?

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Abstract

Background

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.

Methods and Results

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.

Discussion

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.

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