Histopathological Evaluation of Behçet's Disease and Identification of New Skin Lesions

Behçet's disease (BD) is a multisystemic, relapsing inflammatory disorder with an obscure etiology and pathogenesis. Diagnosis depends on the clinician's ability to identify a group of nonspecific mucocutaneous lesions, which also manifest in a number of other diseases. In recent years, there has been an increase in the studies focusing on the histopathological aspects of Behçet's disease diagnostic mucocutaneous lesions. Their results emphasize the value of histopathology and direct immunofluorescence (DIF) in the differential diagnosis of Behçet's disease.

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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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Neuro-Behçet’s Disease – Clinical Features, Diagnosis and Differential Diagnosis

European Neurological Review ◽

10.17925/enr.2018.13.2.93 ◽

2018 ◽

Vol 13 (2) ◽

pp. 93

Author(s):

Selen Ozyurt ◽

Petros Sfikakis ◽

Aksel Siva ◽

Cris S Constantinescu ◽

◽

...

Keyword(s):

Differential Diagnosis ◽

Behçet’S Disease ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Case Reports ◽

Illustrative Case ◽

Inflammatory Disorder ◽

Behcet's Disease ◽

Multisystem Involvement ◽

The Central Nervous System

Background:Behçet’s disease is a relatively uncommon, inflammatory disorder with characteristic mucocutaneous lesions and multisystem involvement, of unknown aetiology; presumably a vascular autoinflammatory syndrome that develops under combined environmental and genetic influences. As neuro-Behçet’s disease affects the central nervous system in about 10% of cases and in ways that can mimic other neuroinflammatory conditions, awareness of its manifestations, significance, and management is important for neurologists. In March 2017, a mini-symposium at the 11th Congress of Controversies in Neurology in Athens, Greece, was dedicated to specific aspects of Behçet’s and neuro-Behçet’s disease. These included an introduction to Behçet’s disease, pathogenesis and treatment, an overview of its neurological manifestations (neuro-Behçet’s disease) and the differential diagnosis from other neuroinflammatory conditions. Illustrative case reports were used.Objectives:To provide a brief overview of neuro-Behçet’s disease that is informative for clinical neurological practice and that follows the structure of the 2017 mini-symposium.Data sources:Relevant recent comprehensive reviews of the subject and relevant original articles and case reports were provided by each speaker at the mini-symposium. This article contains some of these sources and some additions where necessary to emphasise specific points. References are also provided for more comprehensive recent reviews.Limitations:The mini-symposium was an opportunity for providing a brief update and overview of neuro-Behçet’s disease and to exchange ideas and experience among neurologists. As such, it was found to be helpful, but also limited in scope. This resultant article refers to comprehensive reviews on the topic but is not in itself a comprehensive systematic review.Conclusions:Neuro-Bechet’s disease comprises largely two forms, parenchymal and a non-parenchymal. These manifestations seldom overlap in the same individual and may reflect different pathogenetic mechanisms. The principles of treatment largely follow the principles of treating Bechet’s disease in general, with the mainstay being corticosteroids for exacerbations and immunosuppressive treatments for prevention of exacerbations. One notable exception is cyclosporine, which is typically avoided in neuro-Bechet’s disease. Anti-tumour necrosis factor biologicals play an increasing role in treatment. Distinguishing neuro-Behçet’s disease from other neuroinflammatory conditions, such as multiple sclerosis, is essential for both management and prognostic reasons.

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Behcet’s Disease in Southeast Asia. Clinical Features, Genetic Study, and Review of Recent Treatment

Journal of Clinical Rheumatology and Immunology ◽

10.1142/s2661341721300020 ◽

2021 ◽

pp. 1-15

Author(s):

Worawit Louthrenoo

Keyword(s):

Southeast Asia ◽

Behçet’S Disease ◽

Genetic Study ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Clinical Manifestations ◽

Case Series ◽

Skin Lesions ◽

Inflammatory Disorder ◽

Behcet's Disease

Behçet’s disease (BD) is a chronic systemic inflammatory disorder characterized by recurrent oral and genital ulcers, skin lesions, and ocular inflammations. It also can affect other organs such as blood vessels, nervous, gastrointestinal, and articular systems. The disease has a worldwide distribution, but is more prevalent in countries along the Silk Road. Genetic study has shown that the disease is associated with the presence of human leukocyte antigen (HLA)-B*51/B%51:01. BD is considered an uncommon disease in Southeast Asia, and only a few case series have been reported. This article reviews the clinical manifestations and genetic association of BD in Southeast Asian countries and compares them with those in East Asia, South Asia, and the Middle East. It also updated the treatment of BD, particularly the use of biologic agents.

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Potential Infectious Etiology of Behçet's Disease

Pathology Research International ◽

10.1155/2012/595380 ◽

2012 ◽

Vol 2012 ◽

pp. 1-4 ◽

Cited By ~ 15

Author(s):

Massimiliano Galeone ◽

Roberta Colucci ◽

Angelo Massimiliano D'Erme ◽

Silvia Moretti ◽

Torello Lotti

Keyword(s):

Behçet’S Disease ◽

Behcet’S Disease ◽

Molecular Mimicry ◽

Behçet's Disease ◽

Parvovirus B19 ◽

Infectious Agent ◽

Skin Lesions ◽

Inflammatory Disorder ◽

Behcet's Disease ◽

Varicella Zoster

Behçet's disease is a multisystem inflammatory disorder characterized by recurrent oral aphthous ulcers, genital ulcers, uveitis, and skin lesions. The cause of Behçet's disease remains unknown, but epidemiologic findings suggest that an autoimmune process is triggered by an environmental agent in a genetically predisposed individual. An infectious agent could operate through molecular mimicry, and subsequently the disease could be perpetuated by an abnormal immune response to an autoantigen in the absence of ongoing infection. Potentia bacterial are Saccharomyces cerevisiae, mycobacteria, Borrelia burgdorferi, Helicobacter pylori, Escherichia coli, Staphylococcus aureus, and Mycoplasma fermentans, but the most commonly investigated microorganism is Streptococcus sanguinis. The relationship between streptococcal infections and Behçet's disease is suggested by clinical observations that an unhygienic oral condition is frequently noted in the oral cavity of Behçet's disease patients. Several viral agents, including herpes simplex virus-1, hepatitis C virus, parvovirus B19, cytomegalovirus, Epstein-Barr virus and varicella zoster virus, may also have some role.

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Differential Diagnosis: Intestinal Behçet’s Disease

Atlas of Inflammatory Bowel Diseases ◽

10.1007/978-3-642-39423-2_3 ◽

2014 ◽

pp. 53-69

Author(s):

Jae Jun Park ◽

Jae Hee Cheon ◽

Won Ho Kim

Keyword(s):

Differential Diagnosis ◽

Behçet’S Disease ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Intestinal Behçet’S Disease ◽

Behcet's Disease ◽

Intestinal Behcet’S Disease ◽

Intestinal Behçet's Disease

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Pulmonary embolism in Behcet’s disease: a case report

Monaldi Archives for Chest Disease ◽

10.4081/monaldi.2020.1347 ◽

2020 ◽

Vol 90 (4) ◽

Author(s):

Despoina Moumtzi ◽

Marianna Kakoura

Keyword(s):

Pulmonary Embolism ◽

Differential Diagnosis ◽

Behçet’S Disease ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Rare Complication ◽

Pulmonary Involvement ◽

Unknown Etiology ◽

Disease Exacerbation ◽

Behcet's Disease

Behcet’s disease (BD) is a vasculitis of unknown etiology. It is often correlated with thrombophilic factors such as V Leiden. Pulmonary involvement is reported in 1-10% of patients. The most common manifestations are pulmonary aneurysms while pulmonary embolism is a rare complication. A 41-year old man with BD and V Leiden heterozygosity complained of pleurodynia and fever. Pleurodynia deteriorated in the following days and PE was confirmed by CT angiography, without the presence of aneurysms. After the exclusion of the antiphospholipid syndrome, a therapeutic dose of apixaban was initiated. Two weeks later, pleurodynia relapsed in combination with pleural effusion unilaterally. These findings were attributed to disease exacerbation. For this reason, we decided to enhance the immunosuppressive therapy. Six months later, CTPA showed complete remission of the clots. Vasculitis predisposes to thrombosis with or without coexisting thrombophilia. Clinicians should include them in their differential diagnosis and provide personalized treatment, based on immunosuppressants.

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