The prevalence is variable according to ethnic groups and regions in the world: it is more common in the North of Israel, Turkey and the Middle-East (20-420/100,000). Systemic autoimmune disease considered as a vasculitis of unknown cause (promoting factor: HLA B51 ?). The affection begins usually between 20 and 40 years of age but pediatric cases have been reported. 

It is characterized by recurrent episodes of oral and/or genital painful ulcerations and  systemic manifestations:

-         cutaneous: hypodermic nodules (erythema nodosum), necrotic pseudofolliculitis, abnormal reaction to skin trauma (pathergy)

-         ocular: very aggressive anterior and posterior uveitis  (risk of blindness)

-         articular: arthritis in large joints

-         digestive: ulcerative lesions at the level of the digestive tract

-         vascular: thrombosis, arterial aneurysms (including at the level of the pulmonary arteries), Budd Chiari  syndrome (see this term), pericarditis, myocarditis

-         neurological: meningitis or aseptic meningoencephalitis (risk of encephalopathy with or without intracranial hypertension).

Treatment: local for cutaneous and mucosal lesions; in case of systemic signs: corticosteroids, immunosuppressants (azathioprine, cyclosporine...), colchicine, interferon, TNF antagonists ?

Anesthetic implications: 

Depending on the location of lesions. Check renal function (chronic use of NSAIDs and colchicine, immunosuppression) and total blood count. Risk of difficult intubation (especially small  mouth opening) in case of oropharyngeal scarring. Limit the number of skin punctures because each may give rise to skin lesions with ulceration. Oral lesions have been described after a difficult intubation; careful use of the laryngeal mask?

Drug interactions with treatment: colchicine, thalidomide, corticosteroids, immunosuppressants.

References : 

-        Salihoglu Z, Dikmen Y, Demiroluk S, Hamzaoglu I, Paksoy M. 
Oral aphtous ulcers after difficult intubation in a patient with Behcet’s disease. 
Anaesthesia 2002; 57: 620-1

-         Singer O. 
Cogan and Behçet syndromes. 
Rheum Dis Clin N Am 2015; 41: 75-91.

-         Bhalerao PM, Patil VH, Page ND. 
A case of Behcet’s disease posted for surgery: anaesthetic implications. 
Ind J Anaesth 2015; 59: 517-9.

Updated: November 2019