Drug-resistant bullous pemphigoid and inflammatory bowel disease in a pediatric case successfully treated by plasma exchange and extracorporeal photochemotherapy.
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.
Tripodi
Gino
G
Immunohematology and Transfusion Medicine Service, IRCCS G. Gaslini, Genoa, Italy. ginotripodi@ospedale-gaslini.ge.it
Risso
Marco
M
Tenerini
Loredana
L
Gandullia
Paolo
P
Castellano
Emanuela
E
Rivabella
Lucia
L
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