PMID- 34984113 OWN - NLM STAT- PubMed-not-MEDLINE LR - 20220107 IS - 2168-8184 (Print) IS - 2168-8184 (Electronic) IS - 2168-8184 (Linking) VI - 13 IP - 11 DP - 2021 Nov TI - Combination Therapy of Plasma Exchange and Rituximab to Treat Cicatricial Pemphigoid and Bullous Pemphigoid. PG - e19932 LID - 10.7759/cureus.19932 [doi] LID - e19932 AB - The pemphigoid group of subepidermal autoimmune blistering diseases can affect both cutaneous and mucosal tissues. Therapy of this group of diseases, including cicatricial pemphigoid (CP) and bullous pemphigoid (BP), consists of systemic steroids and immunomodulatory agents. Recalcitrant cases have typically been treated with plasmapheresis or rituximab individually. This report describes two patients with severe, rapidly progressive CP and BP refractory to high-dosage systemic steroids and immunomodulatory agents. Both patients were treated with a combination of plasmapheresis and rituximab. In addition to these cases, one retrospective study showed the effectiveness of other immunosuppressants in combination with plasmapheresis in 17 patients with pemphigus refractory to corticosteroids and immunosuppressants alone. No major adverse events occurred in the study. Similar studies employing immunoadsorption and rituximab with various combinations of intravenous immune globulin (IVIg), corticosteroids, and other conventional immunosuppressants have shown promising results in other autoimmune blistering diseases. The successful response in the patients described here, as well as those described in the literature who underwent similar management, provides a possible combination treatment option for patients with severe, recalcitrant pemphigoid. A further trial with a larger group of pemphigoid patients is warranted. CI - Copyright (c) 2021, Kunadia et al. FAU - Kunadia, Anuj AU - Kunadia A AD - Dermatology, University of Central Florida College of Medicine, Orlando, USA. FAU - Sami, Naveed AU - Sami N AD - Dermatology, University of Central Florida College of Medicine, Orlando, USA. LA - eng PT - Case Reports DEP - 20211127 PL - United States TA - Cureus JT - Cureus JID - 101596737 PMC - PMC8713951 OTO - NOTNLM OT - autoimmune blistering diseases OT - bullous pemphigoid OT - cicatricial pemphigoid OT - plasma exchange OT - rituximab COIS- The authors have declared that no competing interests exist. EDAT- 2022/01/06 06:00 MHDA- 2022/01/06 06:01 PMCR- 2021/11/27 CRDT- 2022/01/05 05:56 PHST- 2021/09/11 00:00 [received] PHST- 2021/11/27 00:00 [accepted] PHST- 2022/01/05 05:56 [entrez] PHST- 2022/01/06 06:00 [pubmed] PHST- 2022/01/06 06:01 [medline] PHST- 2021/11/27 00:00 [pmc-release] AID - 10.7759/cureus.19932 [doi] PST - epublish SO - Cureus. 2021 Nov 27;13(11):e19932. doi: 10.7759/cureus.19932. eCollection 2021 Nov.