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]

Search Keywords:
Behçets Disease, Behçets, BD, neutrophilic dermatoses, ERAP1, HLA-B51, CCR1, KLRC4, STAT4, Silk Road, adult, adult onset, adult-onset, adulthood,silk road disease, aphthous ulcers, genital ulcers, mouth sores, genital sores, genital lesions, mouth lesions, oral ulcers, pathergy, pseudofolliculitis, erythema nodosum-like, acneiform nodules, erythema nodosum, Neuro-Behçets, pain, headache, aseptic meningitis, meningoencephalitis, seizures, hemiplegia, cranial nerve palsies. Cerebral venous thrombosis, anterior uveitis, posterior uveitis, uveitis, cataracts, retinal vasculitis, vasculitis, myocarditis, endocarditis, aortic insufficiency, mitral insufficiency, arterial aneurysm, pulmonary embolism, hepatomegaly, splenomegaly or hepatosplenomegaly, abdominal pain, anorexia, diarrhea, bloody diarrhea, lymphadenopathy, arthralgias, arthritis, venous thrombosis, cryoglobulinemia, factor VIII, fibrinolysis, enlarged liver, enlarged spleen, enlarged live and spleen, joint pain, pain,


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Photo credit:

Diepgen TL, Yihune G et al. Behçets Disease. Dermatology Online Atlas. DermIS Dermatology Information System. http://www.dermis.net/dermisroot/en/17101/image.htm

Resources:

The American Behçets Disease Association has some helpful materials at Behcets.com.

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