Liver transplant in a patient with acquired epidermolysis bullosa and associated end-stage liver disease

We report the first case of a liver transplant in a patientwith epidermolysis bullosa acquisita and associatedhepatitis B virus-hepatitis D virus cirrhosis and itsinherent technical issues. Epidermolysis bullosaacquisita is an autoimmune multisystem disorderinvolving skin and mucosa characterized by...

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Bibliographic Details
Published in:Experimental and clinical transplantation 2017-06, Vol.15 (3), p.366-368
Main Authors: Vennarecci,Giovanni, Miglioresi,Lucia, Guglielmo,Nicola, Pelle,Fabio, Santoro,Roberto, Andreuccetti,Jacopo, Ceribelli,Cecilia, Stella,Pietro, Angelo,Corrado, Ettorre,Giuseppe Maria
Format: Article
Language:English
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Summary:We report the first case of a liver transplant in a patientwith epidermolysis bullosa acquisita and associatedhepatitis B virus-hepatitis D virus cirrhosis and itsinherent technical issues. Epidermolysis bullosaacquisita is an autoimmune multisystem disorderinvolving skin and mucosa characterized by theappearing of blisters and erosions. The more severeforms may result in nutritional compromise, anemia,osteopenia, dilated cardiomyopathy, laryngealmucosal involvement, esophageal strictures, bladder,and kidney involvement requiring surgical inter­vention. Epidermolysis bullosa acquisita has becomerecognized as a multisystem disorder that posesseveral surgical challenges. This case shows that livertransplant is a feasible procedure in patients affectedby epidermolysis bullosa acquisita. Patients withepidermolysis bullosa acquisita require a particularpretransplant assessment and a dedicated intra- andpostoperative management of every invasiveprocedure that can traumatize the skin and mucosalepithelium to achieve an uneventful liver transplant.Epidermolysis bullosa acquisita does not represent acontraindication to liver transplant, and immuno­suppression after transplant may favor a goodsystemic control of this immunologic disorder.
ISSN:1304-0855
2146-8427
DOI:10.6002/ect.2015.0018