Discussion
Behcet’s disease (BD) is a rare, systemic disorder initially described by Hulusi Behcet, a Turkish dermatologist, as a triad of uveitis and recurrent oral and genital ulcers. It is prevalent in people of Mediterranean and Middle East countries and less frequent in the Indian subcontinent. The disease usually manifests during the 3rd and 4th decade of their life with male predominance (7,8). We present a case report of a male serving soldier from Nepal in his fourth decade of life. Testosterone may play a role in neutrophil and Th-1 cell activation. This could explain why male patients have a more severe case of BD(9).
The disease raises the mortality rate, particularly in young male patients. Large vessel involvement (pulmonary artery aneurysm), neurological involvement, gastrointestinal system involvement, and cardiac involvement are the most common causes of mortality(10). The exact cause of Behcet’s Disease is unknown and is thought to be multifactorial. The MHC class I region, which includes HLA-B*51, contains the strongest genetic risk factor for BD. There is a 5. 78-fold higher chance of getting BD for individuals with the HLA-B*51/B5 allele than those who did not have this gene (11). Other potential factors can be microbial factors as oral aphthous ulcer typically precede the systemic presentations and occurs before every recurrence of the disease. This case was positive for HLA-B51, which demonstrated probable genetic cause for the occurrence of the disease. Although the significance of HLA-B*51 is well established, it is found to be positive in roughly 60% of patients with Bechet disease. HLA-B*51’s role in the genetic predisposition to the Behcet disease is around 12–19% (12).
There is no confirmatory test for diagnosing Behcet’s Disease as the history and clinical picture are often sufficient for the diagnosis. However, diagnostic criteria proposed by an International Study Group are used for research purposes and clinical purposes too. According to International Study Group criteria for Behcet’s Disease (13), there must be Recurrent oral ulcerations (Minor aphthous, major aphthous, or herpetiform ulcerations which recurred at least 3 times in 12 month period). In addition, two of the following criteria must be met: Recurrent genital Ulcerations, Eye lesions( Uveitis, cells in vitreous on slit-lamp examination, or retinal vasculitis), Skin lesions (Erythema nodosum, pseudofolliculitis, papulopustular lesions), and Positive Pathergy test.
The International Criteria for Behcet’s Disease(ICBD)(14) are proposed to assist earlier diagnosis as ISG clinical diagnosis has low sensitivity. According to signs and symptoms, the International Criteria for Behcet’s Disease has a scoring system; 2 points each for ocular lesions, genital apthosis, and oral apthosis. Each point for skin lesions, neurological manifestations, and vascular manifestations, and Positive pathergy test. A score of more than or equals to 4 indicates Behcet’s diagnosis. Our patient had ocular lesions, genital apthosis, oral apthosis, skin lesions, vascular manifestations ( fundal arteritis), and a positive pathergy test; hence ICBD score was calculated to be 8, which is strongly suggestive of Behcet’s Disease.
BD responds well to steroids. When vital organs are affected, a combination of corticosteroids and immunosuppressant medications is recommended. Our patient responded very well to the steroids. Due to relapsing ocular symptoms, he was placed on adalimumab (TNF-α antagonist). TNF-α, a pro-inflammatory cytokine, is involved in the autoimmune response, inflammation induction, and maintenance. Therefore, it becomes a crucial target molecule in the disease’s treatment(15).
Adalimumab was linked to a reduced risk of uveitis aggravation or visual impairment in non-infectious active intermediate, posterior uveitis, and panuveitis in a placebo-controlled phase 3 research involving patients with BD(16).