title: Drug-resistant bullous pemphigoid and inflammatory bowel disease in a pediatric case successfully treated by plasma exchange and extracorporeal photochemotherapy.
abstract: Bullous pemphigoid (BP) is an autoimmune skin disease that occurs mainly in elderly patients; onset of BP is rare in childhood. Inflammatory bowel diseases (IBD), by contrast, have a pediatric onset in 25% of presenting cases, requiring expert multidisciplinary management. Here we report a pediatric case of IBD (involving stomach, duodenum, ileum, and colon-rectum) associated with a disseminated form of drug-resistant BP successfully treated by plasma exchange (PEX), extracorporeal photochemotherapy (ECP), and corticosteroid therapy. The addition of PEX and ECP to standard treatment induced no severe side effects, prompted a rapidly achieved complete and long-term remission, and allowed dose tapering of the immunosuppressive drugs over an 18-month follow-up.
authors.affiliation: Immunohematology and Transfusion Medicine Service, IRCCS G. Gaslini, Genoa, Italy. ginotripodi@ospedale-gaslini.ge.it
authors.forename: Gino
authors.initials: G
authors.lastname: Tripodi
authors.forename: Marco
authors.initials: M
authors.lastname: Risso
authors.forename: Loredana
authors.initials: L
authors.lastname: Tenerini
authors.forename: Paolo
authors.initials: P
authors.lastname: Gandullia
authors.forename: Emanuela
authors.initials: E
authors.lastname: Castellano
authors.forename: Lucia
authors.initials: L
authors.lastname: Rivabella
mesh_terms: D002675:Child, Preschool; D004351:Drug Resistance; D005260:Female; D006801:Humans; D015212:Inflammatory Bowel Diseases / Q000628:therapy*; D010391:Pemphigoid, Bullous / Q000628:therapy*; D017893:Photopheresis; D010951:Plasma Exchange; D012074:Remission Induction / Q000379:methods