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PATIENT STORY

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A 38-year-old Middle Eastern man presented to the emergency department complaining of sudden onset of chest pain associated with hemoptysis. Physical examination was notable for swelling in his right lower extremity. A provisional diagnosis of right leg DVT and secondary pulmonary embolism was suspected, and the patient underwent a computed tomography (CT) scan of the chest that revealed a 3-cm arterial aneurysm involving the right pulmonary artery (Figure 84-1). Further questioning revealed the patient had been suffering from recurrent painful oral aphthous ulcers for the past 5 years. He reported that the ulcers recurred around 6 times yearly. He also complained of two scrotal ulcers in the past 2 years. Moreover, the patient reported recurrent bilateral eye redness and pain over the past couple of years that were treated as conjunctivitis; no evaluation by an ophthalmologist was performed. This constellation of clinical symptoms, venous thrombosis, as well as the pulmonary aneurysms lead to the diagnosis of Behçet syndrome (BS)—a relapsing systemic vasculitis syndrome that can be as benign as isolated mucocutaneous lesions or be associated with significant morbidity and mortality as in this case.

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FIGURE 84-1

Computed tomography (CT) scan of the chest of a Behçet syndrome (BS) patient demonstrating an aneurysm of the right pulmonary artery. (Reproduced with permission of Informa HealthCare—Books.)

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INTRODUCTION

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  • BS is a systemic inflammatory vasculitis characterized by recurrent oral aphthous ulcerations, genital ulcers, and skin and ocular lesions.1

  • It is a heterogeneous disease with systemic involvement of cardiovascular, gastrointestinal, renal, and nervous systems.

  • BS is also known as the "Old Silk Route" disease, as the first description of the disease is attributed to Hippocrates in the fifth century BC in the Third Book of Endemic Diseases where he describes a disease characterized by aphthous ulcers endemic in Asia Minor.

  • Hulusi Behçet, a Turkish dermatologist, identified the major manifestations of oral aphthous ulcers, genital ulcers, and recurrent hypopyon uveitis (Figure 84-2) and clumped them into a clinical entity that he called the "triple symptom complex" in 1937.

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FIGURE 84-2

Resolving erythema nodosum in a patient with Behçet syndrome (BS).

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EPIDEMIOLOGY

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  • BS is most commonly encountered in Mediterranean countries and countries of the far east along the old "Silk Route"—an ancient commercial line stretching between the Mediterranean, Middle East, and the Far East.

  • Prevalence is between 20 and 421 per 100,000 adults in Turkey; Japan follows with reported prevalence between 13.5 and 20 cases per 100,000 adults. Prevalence is much lower in Western countries ranging from 0.12 to 0.64 cases per 100,000 adults.

  • A recent epidemiologic study revealed an 18-fold increase in the prevalence of this syndrome in the United States. The causes of this increase are ...

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