Post biologic scenario in pemphigus patients at a tertiary care institution


  • Ramesh A. Department of Dermatology, Madras Medical College, Chennai, Tamil Nadu, India
  • Sampath V. Department of Dermatology, Madras Medical College, Chennai, Tamil Nadu, India
  • Shabari A. Department of Dermatology, Madras Medical College, Chennai, Tamil Nadu, India



Adjuvants, Follow-up, Pemphigus, Rituximab


Background: Rituximab is increasingly used for the treatment of pemphigus. Data derived from single center studies following a uniform treatment protocol are limited. The effect of demography and disease type on treatment response is poorly characterized. Aim of this study was to assess the efficacy, adverse effects of rituximab, adjuvants and follow up in pemphigus patients.

Methods: Author undertook a retrospective review of records of 26 pemphigus patients (pemphigus vulgaris 25 and pemphigus foliaceus 1) who had received rituximab infusion. Oral prednisolone was administered in doses up to 0.5 mg/kg of body weight after infusion and tapered over the next 3-4 months according to the disease activity. However, other immunosuppressive agents such as cyclophosphamide and AZT were continued for one year after clinical remission was achieved.

Results: Complete remission was observed in 23 (88.5%) patients. The mean time to disease control and complete remission was 1.10 and 4.36 months, respectively. Three patients experienced relapse after a mean duration of 26 months. Infectious complications like candidiasis and furunculosis developed in two patients. Two patients had hypotension during infusion.

Conclusions: Rituximab is an effective agent in the treatment of pemphigus and also for a long duration of remission with a lower initial dose of oral prednisolone. Severe side effects were rare.


Kanwar AJ, De D. Pemphigus in India. Indian J Dermatol Venereol Leprol. 2011;77(4):439.

Pasricha JS, Khaitan BK, Raman RS, Chandra M. Dexamethasone-cyclophosphamide pulse therapy for pemphigus. Int J Dermatol. 1995;34(12):875-82.

Salopek TG, Logsetty S, Tredget EE. Anti-CD20 chimeric monoclonal antibody (rituximab) for the treatment of recalcitrant, life-threatening pemphigus vulgaris with implications in the pathogenesis of the disorder. J Am Acad Dermatol. 2002;47(5):785-8.

Heizmann M, Itin P, Wernli M, Borradori L, Bargetzi MJ. Successful treatment of paraneoplastic pemphigus in follicular NHL with rituximab: report of a case and review of treatment for paraneoplastic pemphigus in NHL and CLL. Am J Hematol. 2001;66(2):142-4.

Rituxan Receives FDA Approval to Treat Pemphigus Vulgaris | International Pemphigus Pemphigoid Foundation (IPPF). Available at: Http://Www.Pemphigus.Org/Rituxan-Receives-Fda-Approval-To-Treat-Pemphigus-Vulgaris/. Accessed 20 August 2019.

Lever WF, Schaumburg-Lever G. Immunosuppressants and prednisone in pemphigus vulgaris: therapeutic results obtained in 63 patients between 1961 and 1975. Arch Dermatol. 1977;113(9):1236-41.

Kanwar AJ, Vinay K. Treatment of pemphigus: an Indian perspective. Indian J Dermatol Venereol Leprol. 2014;80(4):285-8.

Kanwar AJ, Tsuruta D, Vinay K, Koga H, Ishii N, Dainichi T, et al. Efficacy and safety of rituximab treatment in Indian pemphigus patients. J Eur Acad Dermatol Venereol JEADV. 2013;27(1):e17-23.

Gota V, Karanam A, Rath S, Yadav A, Tembhare P, Subramanian P, et al. Population pharmacokinetics of RedituxTM, a biosimilar Rituximab, in diffuse large B-cell lymphoma. Cancer Chemother Pharmacol. 2016;78(2):353-9.

Sharma VK, Bhari N, Gupta S, Sahni K, Khanna N, Ramam M, et al. Clinical efficacy of rituximab in the treatment of pemphigus: A retrospective study. Indian J Dermatol Venereol Leprol. 2016;82(4):389.

Londhe PJ, Kalyanpad Y, Khopkar US. Intermediate doses of rituximab used as adjuvant therapy in refractory pemphigus. Indian J Dermatol Venereol Leprol. 2014;80(4):300.

Khopkar US, Chadha AA, Nitya MN, Lahoria V. Follow-up data of rituximab as adjuvant therapy in pemphigus. Indian J Dermatol. 2017;62(6):671.

Ahmed AR, Shetty S. A comprehensive analysis of treatment outcomes in patients with pemphigus vulgaris treated with rituximab. Autoimmun Rev. 2015;14(4):323-31.

Kanwar AJ, Vinay K, Sawatkar GU, Dogra S, Minz RW, Shear NH, et al. Clinical and immunological outcomes of high- and low-dose rituximab treatments in patients with pemphigus: a randomized, comparative, observer-blinded study. Br J Dermatol. 2014;170(6):1341-9.

Wang HH, Liu CW, Li YC, Huang YC. Efficacy of rituximab for pemphigus: a systematic review and meta-analysis of different regimens. Acta Derm Venereol. 2015;95(8):928-32.

Vinay K, Kanwar AJ, Sawatkar GU, Dogra S, Ishii N, Hashimoto T. Successful use of rituximab in the treatment of childhood and juvenile pemphigus. J Am Acad Dermatol. 2014;71(4):669-75.

Bystryn JC, Rudolph JL. Pemphigus. Lancet Lond Engl. 2005;366(9479):61-73.

Vinay K, Kanwar AJ, Mittal A, Dogra S, Minz RW, Hashimoto T. Intralesional Rituximab in the Treatment of Refractory Oral Pemphigus Vulgaris. JAMA Dermatol. 2015;151(8):878-82.

Kanwar AJ, Vinay K, Heelan K, Walsh S, Shear NH, Dhiman RK. Use of rituximab in pemphigus patients with chronic viral hepatitis: Report of three cases. Indian J Dermatol Venereol Leprol. 2014;80(5):422.

Albers LN, Liu Y, Bo N, Swerlick RA, Feldman RJ. Developing biomarkers for predicting clinical relapse in pemphigus patients treated with rituximab. J Am Acad Dermatol. 2017;77(6):1074-82.

Bhattacharjee R, De D, Handa S, Minz RW, Saikia B, Joshi N. Assessment of the Effects of Rituximab Monotherapy on Different Subsets of Circulating T-Regulatory Cells and Clinical Disease Severity in Severe Pemphigus Vulgaris. Dermatol Basel Switz. 2016;232(5):572-7.

Colliou N, Picard D, Caillot F, Calbo S, Le Corre S, Lim A, et al. Long-term remissions of severe pemphigus after rituximab therapy are associated with prolonged failure of desmoglein B cell response. Sci Transl Med. 2013;5(175):175ra30.

Joly P, Maho-Vaillant M, Prost-Squarcioni C, Hebert V, Houivet E, Calbo S, et al. First-line rituximab combined with short-term prednisone versus prednisone alone for the treatment of pemphigus (Ritux 3): a prospective, multicentre, parallel-group, open-label randomised trial. Lancet Lond Engl. 2017;389(10083):2031-40.

Hertl M, Jedlickova H, Karpati S, Marinovic B, Uzun S, Yayli S, et al. Pemphigus. S2 Guideline for diagnosis and treatment--guided by the European Dermatology Forum (EDF) in cooperation with the European Academy of Dermatology and Venereology (EADV). J Eur Acad Dermatol Venereol JEADV. 2015;29(3):405-14.

Cho HH, Jin S-P, Chung JH. Clinical experiences of different dosing schedules of rituximab in pemphigus with various disease severities. J Eur Acad Dermatol Venereol JEADV. 2014;28(2):186-91.

Vinay K, Cazzaniga S, Amber KT, Feldmeyer L, Naldi L, Borradori L. Rituximab as first-line adjuvant therapy for pemphigus: Retrospective analysis of long-term outcomes at a single center. J Am Acad Dermatol. 2018;78(4):806–8.

Murrell DF, Peña S, Joly P, Marinovic B, Hashimoto T, Diaz LA, et al. Diagnosis and Management of Pemphigus: recommendations by an International Panel of Experts. J Am Acad Dermatol. 2018.

Ahmed AR, Spigelman Z, Cavacini LA, Posner MR. Treatment of pemphigus vulgaris with rituximab and intravenous immune globulin. N Engl J Med. 2006;355(17):1772-9.

Recommendations of the Advisory Committee on Immunization Practices (ACIP): use of vaccines and immune globulins for persons with altered immunocompetence. MMWR Recomm Rep Morb Mortal Wkly Rep Recomm Rep. 1993;42(RR-4):1-18.

Laniosz V, Lehman JS, Poland GA, Wetter DA. Literature-based immunization recommendations for patients requiring immunosuppressive medications for autoimmune bullous dermatoses. Int J Dermatol. 2016;55(6):599-607.

Leshem YA, Hodak E, David M, Anhalt GJ, Mimouni D. Successful treatment of pemphigus with biweekly 1-g infusions of rituximab: a retrospective study of 47 patients. J Am Acad Dermatol. 2013;68(3):404-11.

Gregoriou S, Giatrakou S, Theodoropoulos K, Katoulis A, Loumou P, Toumbis-Ioannou E, et al. Pilot study of 19 patients with severe pemphigus: prophylactic treatment with rituximab does not appear to be beneficial. Dermatol Basel Switz. 2014;228(2):158-65.

Kanwar AJ, Vinay K. Rituximab in pemphigus. Indian J Dermatol Venereol Leprol. 2012;78(6):671-6.

Schoergenhofer C, Schwameis M, Firbas C, Bartko J, Derhaschnig U, Mader RM, et al. Single, very low rituximab doses in healthy volunteers - a pilot and a randomized trial: implications for dosing and biosimilarity testing. Sci Rep. 2018;8(1):124.

Alaibac M. Ultra-Low Dosage Regimen of Rituximab in Autoimmune Blistering Skin Conditions. Front Immunol. 2018 Available at: Accessed 6 September 2019.

Musette P, Bouaziz JD. B Cell Modulation Strategies in Autoimmune Diseases: New Concepts. Front Immunol. 2018;9:622.

De A, Ansari A, Sharma N, Sarda A. Shifting focus in the therapeutics of immunobullous disease. Indian J Dermatol. 2017;62(3):282.

Pollmann R, Schmidt T, Eming R, Hertl M. Pemphigus: a Comprehensive Review on Pathogenesis, Clinical Presentation and Novel Therapeutic Approaches. Clin Rev Allergy Immunol. 2018;54(1):1-25.

Ellebrecht CT, Choi EJ, Allman DM, Tsai DE, Wegener WA, Goldenberg DM, et al. Subcutaneous veltuzumab, a humanized anti-CD20 antibody, in the treatment of refractory pemphigus vulgaris. JAMA Dermatol. 2014;150(12):1331-5.

Ellebrecht CT, Bhoj VG, Nace A, Choi EJ, Mao X, Cho MJ, et al. Reengineering chimeric antigen receptor T cells for targeted therapy of autoimmune disease. Science. 2016;353(6295):179-84.






Original Research Articles