title: Liver Transplant in a Patient With Acquired Epidermolysis Bullosa and Associated End-Stage Liver Disease.
abstract: We report the first case of a liver transplant in a patient with epidermolysis bullosa acquisita and associated hepatitis B virus-hepatitis D virus cirrhosis and its inherent technical issues. Epidermolysis bullosa acquisita is an autoimmune multisystem disorder involving skin and mucosa characterized by the appearing of blisters and erosions. The more severe forms may result in nutritional compromise, anemia, osteopenia, dilated cardiomyopathy, laryngeal mucosal involvement, esophageal strictures, bladder, and kidney involvement requiring surgical intervention. Epidermolysis bullosa acquisita has become recognized as a multisystem disorder that poses several surgical challenges. This case shows that liver transplant is a feasible procedure in patients affected by epidermolysis bullosa acquisita. Patients with epidermolysis bullosa acquisita require a particular pretransplant assessment and a dedicated intra- and postoperative management of every invasive procedure that can traumatize the skin and mucosal epithelium to achieve an uneventful liver transplant. Epidermolysis bullosa acquisita does not represent a contraindication to liver transplant, and immunosuppression after transplant may favor a good systemic control of this immunologic disorder.
authors.affiliation: From the Department of General Surgery and Organ Transplant, San Camillo Hospital, Rome, Italy.
authors.forename: Giovanni
authors.initials: G
authors.lastname: Vennarecci
authors.forename: Lucia
authors.initials: L
authors.lastname: Miglioresi
authors.forename: Nicola
authors.initials: N
authors.lastname: Guglielmo
authors.forename: Fabio
authors.initials: F
authors.lastname: Pelle
authors.forename: Roberto
authors.initials: R
authors.lastname: Santoro
authors.forename: Jacopo
authors.initials: J
authors.lastname: Andreuccetti
authors.forename: Cecilia
authors.initials: C
authors.lastname: Ceribelli
authors.forename: Pietro
authors.initials: P
authors.lastname: Stella
authors.forename: Corrado
authors.initials: C
authors.lastname: Angelo
authors.forename: Giuseppe Maria
authors.initials: GM
authors.lastname: Ettorre
mesh_terms: D058625:End Stage Liver Disease / Q000175:diagnosis / Q000276:immunology / Q000601:surgery* / Q000821:virology; D016107:Epidermolysis Bullosa Acquisita / Q000150:complications* / Q000175:diagnosis / Q000276:immunology; D005260:Female; D006509:Hepatitis B / Q000150:complications* / Q000175:diagnosis / Q000276:immunology / Q000821:virology; D003699:Hepatitis D / Q000150:complications* / Q000175:diagnosis / Q000276:immunology / Q000821:virology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D016031:Liver Transplantation / Q000009:adverse effects; D008875:Middle Aged; D012307:Risk Factors; D016896:Treatment Outcome
chemical_list: D007166:Immunosuppressive Agents