A Case of HLA-B51 Positive Mucocutaneous Variant of Behçet’s Disease in a Young Indian Male: A Case Report

Background: Behçet’s disease is a rare, systemic inflammatory disorder of unknown etiology affecting the mucocutaneous, vascular, skeletal, ophthalmic, gastrointestinal, and neurological systems. While the exact etiopathogenesis of Behçet’s disease is yet to be established, numerous studies have supported a strong possibility of underlying genetic factors. It is comparatively more common in Turkey, the Middle East, and Mediterranean regions, and only a few cases have been reported from the Indian subcontinent so far. Although several immunological and genetic associations have been suggested, the diagnosis of Behçet’s disease remains primarily clinical and of exclusion. Case Report: In this report, we describe the case of a 22-year-old Indian male who developed multiple aphthous ulcers over his tonsillar pillars and pharyngeal wall following an episode of acute tonsillopharyngitis. Over the course of the next few days, he reported the presence of a genital ulcer and papulopustular lesions over his chest, back and face, eventually prompting a diagnosis of Behçet’s disease. He was treated with oral colchicine and was found to be in remission during a six-week follow-up. Conclusion: This case emphasizes the importance of awareness regarding Behçet’s disease among clinicians in India and warrants further studies on the epidemiology, immunopathogenesis, and management protocols of Behçet’s disease, especially in this country for a better understanding of its prevalence, manifestations, and disease course.

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Immunopathogenesis of Ocular Behçet’s Disease

Journal of Immunology Research ◽

10.1155/2014/653539 ◽

2014 ◽

Vol 2014 ◽

pp. 1-13 ◽

Cited By ~ 11

Author(s):

Un Chul Park ◽

Tae Wan Kim ◽

Hyeong Gon Yu

Keyword(s):

Behçet’S Disease ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Retinal Vasculitis ◽

Skin Lesions ◽

Unknown Etiology ◽

Ocular Involvement ◽

Inflammatory Disorder ◽

General Hypothesis ◽

Behcet's Disease

Behçet’s disease (BD) is a chronic recurrent systemic inflammatory disorder of unknown etiology characterized by oral and genital ulcerations, skin lesions, and uveitis. The ocular involvement of BD, or Behçet’s uveitis (BU), is characterized by panuveitis or posterior uveitis with occlusive retinal vasculitis and tends to be more recurrent and sight threatening than other endogenous autoimmune uveitides, despite aggressive immunosuppression. Although pathogenesis of BD is unclear, researches have revealed that immunological aberrations may be the cornerstone of BD development. General hypothesis of BD pathogenesis is that inflammatory response is initiated by infectious agents or autoantigens in patients with predisposing genetic factors and perpetuated by both innate and acquired immunity. In addition, a network of immune mediators plays a substantial role in the inflammatory cascade. Recently, we found that the immunopathogenesis of BU is distinct from other autoimmune uveitides regarding intraocular effector cell profiles, maturation markers of dendritic cells, and the cytokine/chemokine environment. In addition, accumulating evidence indicates the involvement of Th17 cells in BD and BU. Recent studies on genetics and biologics therapies in refractory BU also support the immunological association with the pathogenesis of BU. In this review, we provide an overview of novel findings regarding the immunopathogenesis of BU.

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A Darwinian view of Behçet's disease

Rheumatology and Immunology Research ◽

10.2478/rir-2021-0013 ◽

2021 ◽

Vol 2 (2) ◽

pp. 91-99

Author(s):

Rhodri Smith ◽

Robert J. Moots ◽

Mariam Murad ◽

Graham R. Wallace

Keyword(s):

Behçet’S Disease ◽

Complex Disease ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Clinical Findings ◽

Unknown Etiology ◽

Inflammatory Disorder ◽

Protective Mechanisms ◽

Behcet's Disease ◽

Cns Lesions

Abstract Behçet’s disease (BD) is a multisystem inflammatory disorder of unknown etiology, characterized by oral and genital ulceration, with other complications including eye, skin, joint, and central nervous system (CNS) lesions. Diagnosis is based on clinical findings, which may differ between patients. There is a strong genetic basis for BD; however, only a few genes have been associated with the disease across the geographical spread of BD. In this article, we discuss the history and combination of genes involved in this complex disease in relation to the geographical range and present our view that the disease has developed from a Darwinian perspective, with different gene polymorphisms that affect the same biological pathway. Moreover, these mutations individually are protective mechanisms against the disease relevant to each region, which affected both archaic and modern humans.

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Treatment of severe generalized chronic periodontitis in a patient with Behçet’s disease: A case report

Journal of International Medical Research ◽

10.1177/0300060518762267 ◽

2018 ◽

Vol 46 (5) ◽

pp. 2037-2045

Author(s):

Satoru Morikawa ◽

Takehito Ouchi ◽

Seiji Asoda ◽

Nobuyuki Horie ◽

Kazuyuki Tsunoda ◽

...

Keyword(s):

Case Report ◽

Behçet’S Disease ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Unknown Etiology ◽

Periodontal Surgery ◽

Behcet's Disease ◽

Dental Biofilm ◽

Periodontal Tissues ◽

Oral Ulcers

Behçet’s disease is a systemic disorder of unknown etiology. It involves multiple organ systems and is characterized by recurring episodes of oral ulcers as well as ocular, genital, and skin lesions. Oral ulcers can affect tooth brushing and impair proper oral hygiene. As a result, a dental biofilm accumulates, and the condition of the teeth and periodontal tissue deteriorates. The aim of this case report is to highlight the efficacy of periodontal treatment for patients with Behçet’s disease. A 51-year-old man with Behçet’s disease presented with generalized severe periodontitis. After basic treatment of the periodontal tissues, periodontal surgery was performed at several sites with bony defects. However, the patient developed severe stomatitis in the oral mucosa and gingiva after periodontal surgery. Administration of the antimicrobial agent cefdinir had little effect on recovery; however, subsequent administration of sitafloxacin resulted in significant improvement of the stomatitis. This case demonstrates that periodontal therapy is very useful for alleviating the oral signs and symptoms of Behçet’s disease. Systemic antibiotic treatment with sitafloxacin (but not cefdinir) and mechanical debridement were effective in preventing the recurrence of aphthous ulcer outbreaks after periodontal surgery.

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An Unusual Case of Motor and Vocal Tics With Obsessive-Compulsive Symptoms in a Young Adult With Behçet's Disease

CNS Spectrums ◽

10.1017/s1092852900022501 ◽

2002 ◽

Vol 7 (12) ◽

pp. 878-881 ◽

Cited By ~ 3

Author(s):

Cathy Budman ◽

Adnan Sarcevic

Keyword(s):

Behçet’S Disease ◽

Unusual Case ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Unknown Etiology ◽

Inflammatory Disorder ◽

Obsessive Compulsive ◽

Obsessive Compulsive Symptoms ◽

Behcet's Disease ◽

Vocal Tics

ABSTRACTBehçet's disease is a chronic relapsing-remitting, multi-system inflammatory disorder of unknown etiology, characterized by recurrent oral and genital ulcers, uveitis, and skin lesions. The most common central nervous system findings are demyelization and perivascular cell infiltration; menin-goencephalitis, cerebral atrophy, and vascular thrombosis may also occur. Neuropsychiatric symptoms may include: paroxysmal dystonia, parkinsonism, delirium, hallucinations, and behavioral disturbances. We report an unusual case of explosive onset of motor and vocal tics with obsessive-compulsive symptoms in a 22-year-old male with Behçet's disease. The putative roles of infection and autoimmune mechanisms in the pathogenesis of tics and obsessive-compulsive symptoms are explored.

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