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

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 five 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.