GİOVANNİ VENNARECCİ, LUCİA MİGLİORESİ, NİCOLA GUGLİELMO, FABİO PELLE, ROBERTO SANTORO, JACOPO ANDREUCCETTİ, CECİLİA CERİBELLİ, PİETRO STELLA, CORRADO ANGELO, GİUSEPPE MARİA ETTORRE
Experimental and Clinical Transplantation - 2017;15(3):366-368
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 inter - vention. 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 immuno - suppression after transplant may favor a good systemic control of this immunologic disorder.
