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Behçet disease
1 OMIM reference -
10 associated genes
67 connected diseases
67 signs/symptoms
Disease Type of connection
Autosomal recessive early-onset inflammatory bowel disease
Pediatric systemic lupus erythematosus
Juvenile rheumatoid factor-negative polyarthritis
Oligoarticular juvenile arthritis
Takayasu arteritis
Blau syndrome
Familial Mediterranean fever
Immunodeficiency due to an early component of complement deficiency
Intermittent hydrarthrosis
Stevens-Johnson syndrome
Primary biliary cirrhosis
Herpetic encephalitis
Mendelian susceptibility to mycobacterial diseases due to complete IL12RB1 deficiency
Budd-Chiari syndrome
Essential thrombocythemia
Familial thrombocytosis
Myelofibrosis with myeloid metaplasia
Polycythemia vera
Autosomal dominant hypohidrotic ectodermal dysplasia
Cerebellar ataxia - hypogonadism
Autosomal dominant hyper-IgE syndrome
Pyogenic bacterial infections due to MyD88 deficiency
Waldenström macroglobulinemia
Hereditary cerebral hemorrhage with amyloidosis, Icelandic type
X-linked lymphoproliferative disease
Autoinflammatory syndrome with pyogenic bacterial infection and amylopectinosis
Congenital analbuminemia
Pulverulent cataract
Pyogenic arthritis - pyoderma gangrenosum - acne
APC-related attenuated familial adenomatous polyposis
Amyotrophic lateral sclerosis
Aneurysm - osteoarthritis syndrome
Anhidrotic ectodermal dysplasia - immunodeficiency - osteopetrosis - lymphedema
Autosomal dominant mendelian susceptibility to mycobacterial diseases due to partial IFNgammaR1 deficiency
Autosomal recessive mendelian susceptibility to mycobacterial diseases due to partial IFNgammaR1 deficiency
Burkitt lymphoma
COG5-CDG
Corneal intraepithelial dyskeratosis with palmoplantar hyperkeratosis and laryngeal dyskeratosis
Desmoid tumor
Familial adenomatous polyposis due to 5q22.2 microdeletion
Familial thoracic aortic aneurysm and aortic dissection
Fibronectin glomerulopathy
Gardner syndrome
Giant cell glioblastoma
Gliosarcoma
Hypohidrotic ectodermal dysplasia with immunodeficiency
Immunodeficiency due to interleukin-1 receptor-associated kinase-4 deficiency
Incontinentia pigmenti
Isolated CoQ-cytochrome C reductase deficiency
MALT lymphoma
Mendelian susceptibility to mycobacterial diseases due to complete IFNgammaR1 deficiency
Papillary or follicular thyroid carcinoma
Precursor T-cell acute lymphoblastic leukemia
Reducing body myopathy
Short stature due to isolated growth hormone deficiency with X-linked hypogammaglobulinemia
Turcot syndrome with polyposis
Vitiligo-associated autoimmune disease
X-linked Emery-Dreifuss muscular dystrophy
X-linked agammaglobulinemia
X-linked mendelian susceptibility to mycobacterial diseases due to IKBKG deficiency
X-linked myopathy with postural muscle atrophy
Familial renal amyloidosis due to Apolipoprotein AII variant
Acute myeloid leukemia with t(8;16)(p11;p13) translocation
Mendelian susceptibility to mycobacterial diseases due to complete IL12B deficiency
Rubinstein-Taybi syndrome due to 16p13.3 microdeletion
Rubinstein-Taybi syndrome due to CREBBP mutations
Translocation renal cell carcinoma
Synonym(s):
(no synonyms)

Classification (Orphanet):
- Rare eye disease
- Rare neurologic disease
- Rare renal disease
- Rare skin disease
- Rare systemic or rheumatologic disease

Classification (ICD10):
- Diseases of the musculoskeletal system and connective tissue -

Epidemiological data:
Class of prevalence: 1-9 / 100 000
Average age onset: variable
Average age of death: adult
Type of inheritance: multigenic/multifactorial
External references:
1 OMIM reference -
1 MeSH reference: D001528

Very frequent
- Arthritis / synovitis / synovial proliferation
- Asthenia / fatigue / weakness
- Enanthema / aphtosa / aphta / leukoplakia
- Facial pain / cephalalgia / migraine
- Fever / chilling
- Follicular / erythematous / edematous papules / milium
- Meningitis / meningeal syndrome
- Myalgia / muscular pain
- Nausea / vomiting / regurgitation / merycism / hyperemesis
- Photophobia
- Subcutaneous nodules / lipomas / tumefaction / swelling
- Testes inflammation / orchitis
- Vascularitis / vasculitides / arteritis

Frequent
- Abnormal gait
- Acne / acnea
- Acute abdominal pain / colic
- Articular / joint pain / arthralgia
- Biological inflammatory syndrome / increased erythrocyte sedimentation rate / CRP
- Gastrointestinal bleeding / hemorrhage / hematemesis / melena / rectorrhagia
- Hemiplegia / diplegia / hemiparesia / limb palsy
- Obnubilation / coma / lethargia / desorientation
- Severe allergic reaction / atopy
- Venous thrombosis / phlebitis / thrombophlebitis
- Vesicles / bullous / exsudative lesions / bullous / cutaneous / mucosal detachment

Occasional
- Abnormal pleura / hydrothorax / pleuresia / pleural effusion / chylothorax
- Angor pectoris / myocardial infarction
- Anorexia
- Aortic valve anomaly / incompetence / insufficiency / regurgitation / bicuspid
- Arterial embolism / thrombosis
- Ataxia / incoordination / trouble of the equilibrium
- Cataract / lens opacification
- Cranial hypertension
- Cranial nerves palsy
- Dizziness
- Early death / lethality
- Encephalitis
- Endocardium anomalies / fibroelastosis / endocarditis
- Gangrena / necrosis
- Hemoptysis
- Hypereflexia
- Intestinal perforation
- Lung / pulmonary infiltrates
- Lymphadenopathy / polyadenopathies
- Malabsorption / chronic diarrhea / steatorrhea
- Mitral valve prolapse / incompetence / insufficiency / regurgitation / ring anomaly
- Myocardium anomalies / myocarditis
- Myositis
- Neuritis / polyneuritis / multineuritis
- Osteonecrosis / bone infarction
- Pancreatitis
- Paresthesia / dysesthesia / hypoesthesia / anesthesia / numbness
- Pericardium anomalies / pericarditis / absence / cysts / pericardial effusion
- Psychic / behavioural troubles
- Psychic / psychomotor regression / dementia / intellectual decline
- Pulmonary thromboembolism
- Pyramidal syndrome
- Renal failure
- Renal glomerular defect / glomerulopathy
- Retinopathy
- Retrobulbar optic neuritis
- Seizures / epilepsy / absences / spasms / status epilepticus
- Splenomegaly
- Transient cerebral ischemia / stroke
- Troubles of memory / amnesia / hypermnesia
- Visual loss / blindness / amblyopia
- Weight loss / loss of appetite / break in weight curve / general health alteration
- Xerophthalmia / dry eyes