Role of Dentists in the Management of Behcet’s Disease: A Case Report

Behcet’s disease (BD) is a multi-system recurrent inflammatory disorder occurring in the form of vasculitis of an unknown etiology. It most frequently affects oral and genital mucosa, skin, eyes, joints, and blood vessels. The definitive diagnosis of BD is based on major symptoms such as recurrent oral and genital ulcers and recurrent skin and ocular lesions, accompanied by symptoms related to various systems. However, early BD manifestations are very similar to recurrent aphthous stomatitis (RAS). Several years from its first appearance are often required for a definitive diagnosis. Objective: To describe a dentist’s role in BD management in a patient with a history of highly recurrent RAS. Case Report: We evaluated a 38-year-old man with a 10-year history of recurrent oral ulcers, accompanied by skin and eyes lesions. His intraoral examination revealed multi-sized ulcers with a yellowishwhite base and regular edges, surrounded by an erythematous halo. Ulcers were located on the tongue, floor of the mouth, and gingival mucosa. Although BD diagnosis was not histopathologically confirmed, the patient’s lesions met the International Criteria for Behcet’s disease with a score of 5. Oral ulcers were managed with chlorhexidine mouthwash 2 times/day and supportive measures. A multidisciplinary approach was used for this patient to provide comprehensive treatment. Conclusion: Dentists can be the first clinicians to detect the possible development of BD in patients with symptoms similar to RAS, but additionally having skin and eye lesions.


INTRODUCTION
Behcet's disease (BD) is a multi-system recurrent inflammatory disorder characterized by the development of vasculitis of an unknown etiology. 1-3 BD affects almost all systems, including the vascular, central nervous, gastrointestinal, pulmonary, and urologic systems and the joints. The disease is characterized by four major symptoms: oral aphthous ulcers, genital ulcers, skin lesions, and ocular lesions. Additionally, one or more minor symptoms can be observed, including arthritis, gastrointestinal ulceration, epididymitis, vascular lesions, and symptoms involving the central nervous system. [3][4][5] In 1937, HÛlusi Behcet, a Turkish dermatologist, was the first to comprehensively describe a triad of symptoms including recurrent oral ulcers, genital ulcers, and uveitis, which were subsequently labeled as Behcet's syndrome. 3,4 BD prevalence is related to an ancient trade route known as the "Silk Road." BD occurs worldwide with marked regional differences. Its highest incidence has been observed in the Mediterranean regions, Middle East, and Far East (Table 1). 3,[6][7][8] The age at onset ranges between 20 and 40. BD more frequently and severely affects males. The male-tofemale ratio differs among countries. Specifically, females are more commonly affected in Japan and Korea, whereas males are more commonly affected in the Middle East region. Severe manifestations such as uveitis and neurological and vascular involvement are predominantly observed in males. 2,7,8 Behcet's disease is a recurrent multisystem vasculitis that can affect any organ or system, but was originally described as orogenital ulcerations and uveitis. Specific criteria have been proposed for diagnosis and identification of affected organs by different national authorities. Behcet's disease is proposed to be due to an antigen/ antibody reaction. The antigen can be external (microbial or other Recurrent aphthous stomatitis (RAS) is a disorder characterized by recurrent ulcers confined to the oral mucosa in patients otherwise asymptomatic. 9 Because recurring oral ulcers are found in BD and RAS, the latter is considered during BD's differential diagnosis. Histopathological examination is necessary for a clinical distinction. 4,5,10 RAS is the most frequent chronic disease of the oral cavity, affecting 5%-25% of the population. It more frequently affects females and individuals of higher socioeconomic levels. 11 The age at onset ranges between 10 and 40 years. BD's definitive diagnosis relies on the presence of major symptoms, occasionally accompanied by minor symptoms. 4,5,11 We report the case of patient with a 10-year history of RAS who then developed skin lesions and eyes symptoms, changing the diagnosis to BD.

CASE REPORT
A 38-year-old male was referred from the Internal Medicine Department for an oral biopsy to identify vasculitis for potential BD diagnosis. He had a 10-year history of RAS (feverless) and later developed skin and eye lesions. These ulcers were painful and selfhealed in 3-4 weeks. Ulcer-free intervals lasted about 2 weeks. As per a dentist's prescription, the patient took triamcinolon acetonide and nystatin oral suspension. In the previous 2 years, the patient suffered from pullulent and painful vesicles of both the thighs. No genital ulcers were reported, whereas itchiness and soreness were present.
A recent complaint of red eyes and blurred vision was diagnosed as uveitis. As a differential diagnosis, an internist requested screening for human immunodeficiency virus (HIV), venereal disease research laboratory (VDRL), Treponema pallidum hemagglutination assay (TPHA), toxoplasma, rubella, cytomegalovirus, and herpes simplex virus (TORCH), and anti-nuclear antibody (ANA). Blood tests showed a low count of leukocytes, basophils, lymphocytes, and neutrophils, with a high erythrocyte sedimentation rate. HIV, VDRL, and TPHA screenings were non-reactive. TORCH examination revealed reactive anti-toxoplasma IgG, anti-cytomegalovirus IgG, and anti-HSV II IgG. ANA examination results were positive, with speckled pattern and a titer of 1/100. A pathergy test was negative, and mycological examination showed absence of fungal infections.
An intraoral examination revealed fair oral hygiene; subgingival and supragingival calculus; and ulcers 2-5 mm in diameter, surrounded by an erythematous halo and yellowish-white base, located at the border between     In this report, oral ulcers were characterized by similar features among each other. Specifically, they were minor aphthous, with a yellowish-white base and surrounded by an erythematous halo and well-circumscribed and round ulcers. These ulcers self-healed and spread to different sites of the oral cavity. Tursen found that histopathological examination of oral ulcers in BD showed nonspecific pathological findings, with lymphocytic vasculitis identified in severe ulcerations. 18 In the present study, BD diagnosis was not confirmed by histopathological examination possibly due to the ulcers' healing process. However, the patient's lesions (oral, eye, and skin lesions) met the ICBD guidelines with a score of 5.
Till date, there is no specific diagnostic laboratory test or histopathological finding for BD. A definitive diagnosis relies on clinical features that requires several years. Patients with exclusively recurrent oral ulcers may not obtain a definitive diagnosis for BD; therefore, these patients require in-depth examination and accurate follow-up, particularly in countries with a high BD prevalence. 16

CONCLUSION
Dentists' role is important in establishing BD diagnosis; they could be the first to detect the disease. Recurrent oral ulcers are a common initial symptom of BD, accompanied by skin and eye lesions. A multidisciplinary approach is required to diagnose and treat the disease.