Treatment with Rituximab in extensive bullous pemphigoid - recalcitrant to conventional treatment: a report of 2 cases

Authors

  • Be Be Safura Department of Dermatology, Maharajah’s Institute of Medical Sciences, Vizianagaram, Andhra Pradesh, India
  • K. V. T. Gopal Department of Dermatology, Maharajah’s Institute of Medical Sciences, Vizianagaram, Andhra Pradesh, India
  • P. V. Krishnam Raju Department of Dermatology, Maharajah’s Institute of Medical Sciences, Vizianagaram, Andhra Pradesh, India
  • B. Rekha Rani Department of Dermatology, Maharajah’s Institute of Medical Sciences, Vizianagaram, Andhra Pradesh, India

DOI:

https://doi.org/10.18203/issn.2455-4529.IntJResDermatol20241724

Keywords:

Pemphigoid diseases, Autoimmune blistering disease, Bullous pemphigoid, Rituximab

Abstract

Bullous pemphigoid (BP) is the most common autoimmune blistering disease requiring treatment with immunosuppressive medications; however, finding a therapy that has a sustained durable response and an acceptable side effect profile has been challenging. Rituximab (RTX) is a monoclonal antibody targeting CD20, a transmembrane protein expressed on B cells, causing B cell depletion. RTX has shown great efficacy in studies of pemphigus vulgaris, but data of pemphigoid diseases are limited. We report a 60 year old male who presented with widespread fluid filled lesions and raw areas preceded by itching since 3 weeks. Dermatological examination revealed multiple intact bullae, vesicles were seen over urticarial plaques and multiple raw areas, erosions with crust formation over trunk, upper and lower extremities. Histopathological examination (HPE) showed sub epidermal blister. Direct immunofluorescence (DIF) revealed IgG: positive (+3), C3: positive (+2), linear pattern, along the dermoepidermal junction. Based on the clinical features, HPE and DIF findings, diagnosis of BP was made. Patient did not respond to high dose of corticosteroids for 3 weeks. Second case was known case of BP who did not respond to conventional treatment even after 5 years. Both cases were treated with Rheumatoid arthritis protocol of RTX and marked improvement was seen after 8 weeks of treatment with no recurrence of lesions. Our experience shows that in all cases of extensive BP and cases not responding to conventional treatment, injection rituximab should be considered for in view of ease of administration, excellent safety profile and good efficacy.

References

Schmidt E, Groves R. Immunobullous Diseases (Bullous Pemphigoid). In: Christopher Griffiths, Jonathan Barker, Tanya Bleiker, Robert Chalmers and Daniel Creamer. Rook's Textbook of Dermatology, 9 th ed. Published by John Wiley & Sons. Ltd. 2016: 50.10-50.22.

Lo Schiavo A, Ruocco E, Brancaccio G, Caccavale S, Ruocco V, Wolf R. Bullous pemphigoid: etiology, pathogenesis, and inducing factors: facts and controversies. Clin Dermatol. 2013;31(4):391-9.

Khandpur S, Verma P. Bullous pemphigoid. Indian J Dermatol Venereol Leprol. 2011;77(4):450-5.

Thoma-Uszynski S, Uter W, Schwietzke S, Hofmann SC, Hunziker T, Bernard P, et al. BP230- and BP180-specific auto-antibodies in bullous pemphigoid. J Invest Dermatol. 2004;122(6):1413-22.

Bernard P, Antonicelli F. Bullous pemphigoid: a review of its diagnosis, associations and treatment. Am J Clin Dermatol. 2017;18(4):513-28.

Kasperkiewicz M, Shimanovich I, Ludwig RJ, Rose C, Zillikens D, Schmidt E. Rituximab for treatment-refractory pemphigus and pemphigoid: a case series of 17 patients. J Am Acad Dermatol. 2011;65(3):552–8.

Polansky M, Eisenstadt R, DeGrazia T, Zhao X, Liu Y, Feldman R. Rituximab therapy in patients with bullous pemphigoid: A retrospective study of 20 patients. J Am Acad Dermatol. 2019;81(1):179-86.

De D, Bishnoi A, Handa S, Mahapatra T, Mahajan R. Effectiveness and safety analysis of rituximab in 146 Indian pemphigus patients: a retrospective single-center review of up to 68 months follow-up. Indian J Dermatol Venereol Leprol. 2020;86(1):39-44.

Shetty S, Ahmed AR. Treatment of bullous pemphigoid with rituximab: critical analysis of the current literature. J Drugs Dermatol. 2013;12(6):672-7.

Lamberts A, Euverman HI, Terra JB, Jonkman MF and Horváth B. Effectiveness and Safety of Rituximab in Recalcitrant Pemphigoid Diseases. Front Immunol. 2018;9:248.

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Published

2024-06-26

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Section

Case Reports