Refractory pemphigus vulgaris and high‐intensity extracorporeal photopheresis: A case report

• Published in: Photodermatology, photoimmunology & photomedicine Vol. 39; no. 3; pp. 296 - 300
• Main Authors: Castillo‐Aleman, Yandy Marx, Bencomo‐Hernandez, Antonio Alfonso, Ventura‐Carmenate, Yendry, Villegas‐Valverde, Carlos Agustin, Rivero‐Jimenez, Rene Antonio, Al Amin, Mariam Thabet, Martinez, May Ann, Cato, Marlene Ponce, Victorino Roque, Jay Mary Rose, Kistan, Jerusha, Othman, Jekhsi, Brown, Sadaf, Al‐Kaabi, Fatema Mohammed
• Format: Journal Article
• Language: English
• Published: England 01.05.2023
• Subjects: autoimmune blistering disorders; case report; extracorporeal photopheresis; immunophenotyping; mass cytometry; pemphigus vulgaris; case report; Extracorporeal photopheresis; mass cytometry; autoimmune blistering disorders; immunophenotyping; pemphigus vulgaris
• ISSN: 0905-4383; 1600-0781
• DOI: 10.1111/phpp.12834

A 41‐year‐old man with oral pemphigus vulgaris (PV) presented to our clinic with a history of no response to numerous immunosuppressant agents and was referred for extracorporeal photopheresis (ECP) therapy. Although the patient underwent a high‐intensity ECP regimen for 5 months, which included two different photopheresis systems, his oral dysesthesia continued to interfere with oral intake, leading to continued weight loss and other adverse events. The intervention was associated with changes in several immune cell subpopulations without modifying the anti‐epidermal antibody titers, aligned with his poor clinical outcome. To the best of the authors' knowledge, this is the first report to examine immunophenotyping of a PV patient who was refractory to previous immunosuppression and recalcitrant to high‐intensity ECP therapy.