Behçets Disease

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Acronym:
BEHÇETS/BD

SAID group:
Idiopathic, Neutrophilic Dermatoses

Gene:
ERAP1 (with HLA-B51); also variants near: CCR1, KLRC4, STAT4. [42] Genetic testing in research studies only. (Article with more info in the Genetic testing button)

Inheritance:
Complex

Ethnicity:
Rare in the USA. More common in the Middle East, Asia and Japan (Silk Road Route). [42] [43]

Frequency:
Prevalence is 80-370:100,000 people in Turkey, 10:100,000 in Japan & 0.6:100,000 in Yorkshire, UK. [3]

Timing of symptoms:
Aphthous ulcers are present in almost all patients during flares, for around 10 days with inflammation in the eye and arthritis. [42] [43]

Age of onset:
Most show symptoms in early adulthood (20s-30s) but the onset can be in childhood, or any age. [42] [43]

Skin cutaneous:
Pathergy. Pseudofolliculitis, erythema nodosum-like and/or acneiform nodules. 98% with aphthous ulcers & 65% have genital ulcers. [43]

Neurologic:
20-40% have Neuro-Behçets with headaches, aseptic meningitis or meningoencephalitis, seizures, hemiplegia, or cranial nerve palsies. Cerebral venous thrombosis with high intracranial pressure (ICP) noted. [43]

Auditory:
Not noted. [42] [43]

Ophthalamic:
Frequent anterior and/or posterior uveitis. Cataracts, retinal vasculitis <30% risk for blindness. Papilledema with neurologic involvement (Neuro-Behçets). [43]

Cardiopulmonary:
Myocarditis, endocarditis with aortic or mitral insufficiency, arterial aneurysm, pulmonary embolism. [43]

Abdominal:
Ulcers from mouth to anus. Some with hepatomegaly, splenomegaly or hepatosplenomegaly. Nausea, abdominal pain, anorexia, diarrhea (may be bloody). [43]

Lymphatic:
Splenomegaly; lymphadenopathy. [43]

Joints bones muscles cartilage:
45% have arthralgias and/or arthritis–often the knees and/or ankles, but other joints can be affected. Joint issues may be the first sign of Behçets. X-ray is normal but the synovium often has high neutrophils or mononuclear cells and a vasculitis process. [43]

Vasculitis:
Extensive vasculitis. 30% with venous thrombosis. [43]

Amyloidosis:
Not noted. [42] [43]

Abnormal labs:
Leukocytosis common. Normal–rarely elevated ESR or CRP. Some cryoglobulinemia, elevated factor VIII, fibrinolysis. [43]

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