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Year : 2022  |  Volume : 67  |  Issue : 6  |  Page : 753-755
Successful treatment of recurrent oral and genital ulcers in Behcet's disease with rituximab

1 Department of Dermatology, Venereology and Leprosy, Sardar Patel Medical College, Bikaner, Rajasthan, India
2 Department of Dermatology, Venereology and Leprosy, GMC, Kota, Rajasthan, India

Date of Web Publication23-Feb-2023

Correspondence Address:
Alpana Mohta
Department of Dermatology, Venereology and Leprosy, Sardar Patel Medical College, Bikaner, Rajasthan
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijd.ijd_531_21

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How to cite this article:
Mohta A, Jain SK, Mehta RD. Successful treatment of recurrent oral and genital ulcers in Behcet's disease with rituximab. Indian J Dermatol 2022;67:753-5

How to cite this URL:
Mohta A, Jain SK, Mehta RD. Successful treatment of recurrent oral and genital ulcers in Behcet's disease with rituximab. Indian J Dermatol [serial online] 2022 [cited 2023 Mar 29];67:753-5. Available from:


Behcet's disease, also known as malignant aphthosis, is a neutrophilic autoinflammatory dermatosis with multisystem vasculitis. This multisystem dermatosis is characterized by recurrent oral, genital ocular lesions coupled with uveitis, and arthritis.[1] Systemic involvement may include renal, neurological, cardiovascular, and gastrointestinal vasculitis. Herein, we report a case of Behcet's disease with recalcitrant oral and genital ulcers successfully managed with Rituximab.

A 34-year-old female patient presented to our outpatient department with a history of recurrent painful oral and genital ulcers for the last 3 years, along with multiple painful erythematous lesions over bilateral upper and lower limbs on and off for the last 7 months [Figure 1]a and [Figure 2]a. The patient was a known case of Behcet's disease with oral and ocular involvement, on and off treatment for the last 2 years with various drugs ranging from systemic steroids and cyclophosphamide to oral colchicine, dapsone, and apremilast, with multiple recurrences and little to no response. On further inquiry, the patient revealed that her oral ulcers were painful but did not bleed and used to keep healing on their own in 10–15 days with the appearance of new lesions continuously. Her ulcers had recurred six times so far in the last year. Meanwhile, the recurrent painful lesions on her limbs, suggestive of erythema nodosum, would last for 2 to 4 weeks before healing and have recurred five to six times so far.
Figure 1: (a) Multiple oral aphthae in Behcet's disease. (b) Complete resolution of oral ulcers after 2 doses of Rituximab

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Figure 2: (a) Multiple genital ulcers over left labia in the same patient. (b) Complete clearance of ulcers after Rituximab therapy

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The patient had a raised erythrocyte sedimentation rate (27 mm/h) and C-reactive protein (11.55 mg/dl). Rest of the investigations, namely hemogram, liver and renal function tests, serology for hepatitis B, hepatitis C and HIV were unremarkable. However, the patient tested positive for HLAB 51 antigen. Pathergy test was also positive. After a thorough analysis to rule out any other systemic illness or infection, the patient was planned for treatment with Rituximab according to rheumatoid arthritis protocol. Pretreatment of the patient was done with tablet paracetamol 500 mg, 22.75 mg injection pheniramine maleate, and 100 mg injection hydrocortisone given 30 minutes before infusion. Then Rituximab was injected using a slow IV pediatric drip set after taking all due precautions. Two doses of 1 gm Rituximab were given one month apart. There was complete healing in all the existing lesions with an appearance of no new lesions or recurrence in the next 1 year [Figure 1]b and [Figure 2]b.

The three phenotype of Behcet's disease include the mucocutaneous with skeletal, extra parenchymal neurological, and parenchymal neurological with the ophthalmic variant.[2] Various immunosuppressives are used for treatment including steroids, methotrexate, cyclophosphamide, mycophenolate, colchicine, azathioprine, dapsone, apremilast, etc. Many biologicals have also been tried including TNF alpha inhibitors like infliximab, adalimumab, and etanercept as well as IL-6 antibodies like tocilizumab with variable success rates [Table 1].[3]
Table 1: Biologicals used in the management of Behcet's Disease

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In Behcet's disease there is an altered antigen response seen in B cells, especially memory B cells. The levels of activated and memory B cells are found to be positively correlated to disease severity.[2] The pioneer study of the efficacy of the safety of Rituximab in Behcet's disease was conducted by Fereydoun.[7] Rituximab acts by inhibiting the entire lineage of B cells containing CD 20 receptors, apart from plasma cells and pre-pro B cells. It also suppresses antigen presentation and cytokinemia, by T-cell inhibition. To date, only a handful of cases have described the use of this novel therapy in the management of neutrophilic dermatoses.[7],[8] Rituximab is especially useful in the management of neurological involvement in Behcet's disease. However, more controlled studies need to be conducted to establish its efficacy consistently.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

   References Top

di Meo N, Bergamo S, Vidimari P, Bonin S, Trevisan G. Analysis of diagnostic criteria in adamantiades-behçet disease: A retrospective study. Indian J Dermatol 2013;58:275-7.  Back to cited text no. 1
Whittam DH, Tallantyre EC, Jolles S, Huda S, Moots RJ, Kim HJ, et al. Rituximab in neurological disease: Principles, evidence and practice. Pract Neurol 2019;19:5-20.  Back to cited text no. 2
Merashli M, Eid RE, Uthman I. A review of current management of vasculo-Behcet's. Curr Opin Rheumatol 2018;30:50-6.  Back to cited text no. 3
McNally TW, Damato EM, Murray PI, Denniston AK, Barry RJ. An update on the use of biologic therapies in the management of uveitis in Behçet's disease: A comprehensive review. Orphanet J Rare Dis 2017;12:130.  Back to cited text no. 4
Caso F, Costa L, Rigante D, Lucherini OM, Caso P, Bascherini V, et al. Biological treatments in Behçet's disease: Byond anti-TNF therapy. Mediators Inflamm 2014;2014:107421.  Back to cited text no. 5
Zouboulis CC. Adamantiades–Behçet disease. In: Tony B, Stephen B, Neil C, Christopher G, editors. 9th ed. Rook's Textbook of Dermatology. Hoboken, NJ: Wiley-Blackwell; 2016. p. 48.1-10.  Back to cited text no. 6
Davatchi F, Shams H, Rezaipoor M, Sadeghi-Abdollahi B, Shahram F, Nadji A, et al. Rituximab in intractable ocular lesions of Behcet's disease; randomized single-blind control study (pilot study). Int J Rheum Dis 2010;13:246-52.  Back to cited text no. 7
Garcia-Estrada C, Casallas-Vanegas A, Zabala-Angeles I, Gomez-Figueroa E, Rivas-Alonso V, Flores-Rivera J. Rituximab as an effective therapeutic option in refractory Neuro-Behçet syndrome. J Neuroimmunol 2020;346:577308.  Back to cited text no. 8


  [Figure 1], [Figure 2]

  [Table 1]


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