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Year : 2021  |  Volume : 14  |  Issue : 6  |  Page : 717-718  

Behcet's disease: A rarity

Department of Oral Medicine and Radiology, Tamil Nadu Government Dental College and Hospital, Affiliated to Tamil Nadu Dr. MGR Medical University, Chennai, Tamil Nadu, India

Date of Submission14-Jul-2020
Date of Decision25-Jul-2020
Date of Acceptance07-Aug-2020
Date of Web Publication30-Apr-2021

Correspondence Address:
S Jayachandran
Department of Oral Medicine and Radiology, Tamil Nadu Government Dental College and Hospital, Affiliated to Tamil Nadu Dr. MGR Medical University, Chennai - 600 003, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/mjdrdypu.mjdrdypu_385_20

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How to cite this article:
Jayachandran S. Behcet's disease: A rarity. Med J DY Patil Vidyapeeth 2021;14:717-8

How to cite this URL:
Jayachandran S. Behcet's disease: A rarity. Med J DY Patil Vidyapeeth [serial online] 2021 [cited 2021 Dec 6];14:717-8. Available from: https://www.mjdrdypv.org/text.asp?2021/14/6/717/315359

Behçet's disease is named after the Turkish dermatologist and scientist Hulusi Behçet who first recognized and reported the three main symptoms associated with the syndrome. Behcet's disease (BD) is a rare systemic vasculitis characterized by oral aphthous ulcers, ocular lesions, genital ulcers, and other systemic manifestations. The etiology of BD is largely unknown and is believed to be due to an autoimmune process. The most consistent genetic marker of Behçet's syndrome is HLA-B51 allele located in the MHC locus, on chromosome 6p.[1] Herpes simplex virus-1 and Streptococcus have been postulated as possible environmental triggers. T cell homeostasis perturbation, especially Th1 and Th17 expansion, and decreased regulation by Tregs are now supposed to be the cornerstone of the pathogenesis.[2] Recurrent oral ulcers represent the earliest disease manifestation in 47%–86% of patients. Oral aphthous ulcer characterized by round to oval disciform ulceration with a white or yellowish necrotic base and surrounded by erythematous halo is the most common clinical feature and is usually the first manifestation.[3] Genital ulceration is the second most common observed feature of BD. Ocular manifestations of BD include nongranulomatous iridocyclitis, chorioretinitis, complicated cataracts, lens pigmentation, chorioretinal atrophy, optic nerve atrophy, and secondary glaucoma.[4] Skin or cutaneous manifestations are also one of the most common features of BD and these include erythema nodosum like eruptions, pseudofolliculitis, and papulopustular lesions. Vascular BD affects both arteries and veins of all sizes and is more common in men than women. Therapy of BD involves benzydamine hydrochloride 0.15% oral rinse (local analgesic), chlorhexidine mouthwash (anti-infective), and lidocaine spray or gel for oral lesions, topical anesthetics (10% lidocaine spray or 2%–5% gel), topical corticosteroid ointment for genital ulcers, steroids, and azathioprine for uveitis. Management approaches with oral corticosteroids, disease-modifying drugs such as azathioprine, tumor necrosis factor-alpha, and interferon-α have been found to be successful.[5]

Idiopathic thrombocytopenic purpura (ITP) is a condition characterized by a low platelet count (thrombocytopenia) of no known cause (idiopathic). The cause appears to be mainly related to antibodies against platelets. Most cases are asymptomatic; however, in some cases, very low platelet counts can lead to a bleeding diathesis and purpura. It is a disorder that affects the overall number of blood platelets rather than their function. The diagnosis of ITP is one of exclusion, which means that the other causes of thrombocytopenia are to be ruled out before arriving at the diagnosis of ITP. The treatment is aimed to provide a safe platelet count to prevent major bleeding and avoid unnecessary treatment of asymptomatic patients with mild-to-moderate thrombocytopenia. Corticosteroids, typically prednisone, are the backbone of the initial treatment. Intravenous immunoglobulin, rituximab (monoclonal antibody), immunosuppressants, dapsone, and other novel agents that increase the platelet count constitute the other management approaches of ITP.[6]

The association of BD with other autoimmune diseases is a scope for further research. In conclusion, the management should follow a multidisciplinary approach involving dermatologists, ophthalmologists, rheumatologists, and oral medicine specialists to resolve the various incapacitating manifestations. This will aid in the proper diagnosis and better treatment planning, which will improve the quality of life of the patients.[7],[8]

  References Top

Yazici H, Fresko I, Yurdakul S. Behçet's syndrome: Disease manifestations, management, and advances in treatment. Nat Clin Pract Rheumatol 2007;3:148-55.  Back to cited text no. 1
Greco A, De Virgilio A, Ralli M, Ciofalo A, Mancini P, Attanasio G, et al. Behçet's disease: New insights into pathophysiology, clinical features and treatment options. Autoimmun Rev 2018;17:567-75.  Back to cited text no. 2
Alpsoy E, Donmez L, Bacanli A, Apaydin C, Butun B. Review of the chronology of clinical manifestations in 60 patients with Behçet's disease. Dermatology 2003;207:354-6.  Back to cited text no. 3
Adeeb F, Stack AG, Fraser AD. Knitting the Threads of Silk through Time: Behçet's Disease-Past, Present, and Future. Int J Rheumatol. 2017;2017:2160610. doi:10.1155/2017/2160610.  Back to cited text no. 4
Nair JR, Moots RJ. Behcet's disease. Clin Med (Lond) 2017;17:71-7.  Back to cited text no. 5
Kayal L, Jayachandran S, Singh K. Idiopathic thrombocytopenic purpura. Contemp Clin Dent 2014;5:410-4.  Back to cited text no. 6
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Sadaksharam J, Preethi M. Multidisciplinary clinical management of paraneoplastic pemphigus – A case report. J Clin Diagn Res 2017;11:ZD01-3.  Back to cited text no. 7
Sadaksharam J. Current Therapeutic Modalities of Immunobullous Lesions. Contemp Clin Dent 2019;10:407-9.  Back to cited text no. 8
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