Elsevier

Clinics in Dermatology

Volume 35, Issue 5, September–October 2017, Pages 421-434
Clinics in Dermatology

Behçet disease: New aspects

https://doi.org/10.1016/j.clindermatol.2017.06.004Get rights and content

Abstract

Behçet disease is currently considered an “autoinflammatory disease” triggered by infection and environmental factors in genetically predisposed individuals. Although the disease is characterized by recurrent oral and genital aphthous ulcers and ocular involvement, it can affect multiple organ systems. Complex aphthosis is characterized by recurrent oral and/or genital aphthous ulcers. It is important to evaluate the patient with complex aphthosis for Behçet disease and related systemic disorders. We discuss the etiopathogenesis, clinical features, diagnostic criteria, and treatment approaches for complex aphthosis and Behçet disease in light of the current literature.

Introduction

Behçet disease (BD) was first described by Hulusi Behçet (1889-1948), a Turkish dermatologist, as “recurrent oral aphthous ulcers, genital ulcers, and ‘hypopyon-uveitis’” in 1937.1 It is a chronic, relapsing systemic disorder characterized by variable clinical manifestations, including oral and genital aphthae, cutaneous lesions, ocular, gastrointestinal, neurologic involvement, and arthritis.2

Recurrent aphthous stomatitis (RAS), also referred to as recurrent aphthous ulcers, is an idiopathic intraoral ulcerative disease that develops in an otherwise healthy individual.3 RAS is divided into two forms: simple aphthosis and complex aphthosis.4 Complex aphthosis is defined to describe patients suffering three or more almost constantly present oral aphthae, or oral and genital aphthae, in the absence of BD.5 Oral and genital ulcers in BD are the most common and often the first clinical manifestations of the disease.[1], [6], [7], [8] The consideration of BD in the differential diagnosis and follow-up of patients presenting with complex aphthosis is, therefore, very important.

Section snippets

Epidemiology

Behçet disease is also known as “Silk Road disease,” and its prevalence in the Mediterranean, Central Asia, and the Far East is significantly higher than in Europe and the United States.[9], [10], [11], [12] The mean prevalence varies between 1 in 1000 and 1 in 10,000.[9], [13] Turkey has the highest prevalence among endemic regions. The prevalence in Turkey has been reported as 20 to 421 in 100,000 in studies conducted in the adult population.[9], [10], [11], [12]

The disease commonly occurs in

Etiopathogenesis

Behçet disease is currently thought to be an autoinflammatory disease triggered by exogenous factors in genetically susceptible individuals.[21], [22], [23] Some investigators believe that complex aphthosis is the incomplete form of BD due to their common etiopathogenetic features.[24], [25] RAS has a different geographic distribution than BD, and the human leukocyte antigen (HLA) genes associated with susceptibility may be different.

Recurrent aphthous stomatitis

The lesions of recurrent aphthous stomatitis are found in 86% to 100% of BD patients and are usually the initial sign.[8], [15], [69] Oral aphthae are also the most common clinical finding in the juvenile age group and constitute the initial sign.70 An increase in oral aphthae prevalence during pregnancy has been emphasized[71], [72]; however, some studies report that oral aphthae do not always accompany any period of the disease in BD patients.[73], [74] The onset of the disease may be later

Diagnosis

Currently no laboratory test can diagnose BD. The diagnosis is usually made according to the diagnostic criteria published by the ISG in 1990. According to these criteria, the disease is diagnosed with the concurrent presence of two of the following findings in addition to oral aphthae: genital ulcers, skin lesions, ocular involvement, and pathergy test positivity80 (Table 2). The International Team for the Revision of the International Criteria for Behçet Disease was established with the

Treatment

The main goals of treatment of BD are to control clinical manifestations, decrease inflammation, suppress the immune system, and prevent secondary organ damage.[115], [116] The choice of treatment varies depending on the involved organs and the severity of the disease, age and gender of the patient, and the duration of the disease.69

Conclusions

Behçet disease is a very complicated entity. Mucocutaneous lesions in BD usually constitute the initial symptom, but ocular, neurologic, vascular, and gastrointestinal involvements are often responsible for major morbidity and mortality. Experience is very important in assessing patients with complex aphthosis for the correct diagnosis. In endemic areas the differentiation between complex aphthosis and BD is less challenging. When BD is highly suspected, a detailed history is helpful.170

New

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