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1 OMIM reference -
10 associated genes
67 signs/symptoms
COMMON GENES: 1
COMMON SIGNS: 17
1 OMIM reference -
1 associated gene
42 signs/symptoms
Behçet disease
Stevens-Johnson syndrome

C4A HLA-B
HLA-B
IL10
IL12RB2
IL23R
MEFV
NOD2
STAT4
TLR4
UBAC2


COMMON
GENES
HLA-B



Citations in the biomedical literature:


Behçet disease
C4A HLA-B IL10 IL12RB2 IL23R MEFV
NOD2 STAT4 TLR4 UBAC2
Stevens-Johnson syndrome



Behçet disease
Stevens-Johnson syndrome

Synonym(s):
(no synonyms)

Synonym(s):
- Dermatostomatitis, Stevens Johnson type

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

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

Epidemiological data:
Class of prevalence: 1-9 / 100 000
Average age onset: variable
Average age of death: adult
Type of inheritance: multigenic/multifactorial
Epidemiological data:
Class of prevalence: unknown
Average age onset: variable
Average age of death: -
Type of inheritance: sporadic

External references:
1 OMIM reference -
1 MeSH reference: D001528
External references:
1 OMIM reference -
1 MeSH reference: D013262


COMMON
SIGNS
- Abnormal pleura / hydrothorax / pleuresia / pleural effusion / chylothorax
- Acute abdominal pain / colic
- Angor pectoris / myocardial infarction
- Asthenia / fatigue / weakness
- Enanthema / aphtosa / aphta / leukoplakia
- Fever / chilling
- Gastrointestinal bleeding / hemorrhage / hematemesis / melena / rectorrhagia
- Intestinal perforation
- Malabsorption / chronic diarrhea / steatorrhea
- Myocardium anomalies / myocarditis
- Nausea / vomiting / regurgitation / merycism / hyperemesis
- Pancreatitis
- Photophobia
- Renal failure
- Vesicles / bullous / exsudative lesions / bullous / cutaneous / mucosal detachment
- Visual loss / blindness / amblyopia
- Weight loss / loss of appetite / break in weight curve / general health alteration


Behçet disease
Stevens-Johnson syndrome

Very frequent
- Arthritis / synovitis / synovial proliferation
- Facial pain / cephalalgia / migraine
- Follicular / erythematous / edematous papules / milium
- Meningitis / meningeal syndrome
- Myalgia / muscular pain
- Subcutaneous nodules / lipomas / tumefaction / swelling
- Testes inflammation / orchitis
- Vascularitis / vasculitides / arteritis

Frequent
- Abnormal gait
- Acne / acnea
- Articular / joint pain / arthralgia
- Biological inflammatory syndrome / increased erythrocyte sedimentation rate / CRP
- Hemiplegia / diplegia / hemiparesia / limb palsy
- Obnubilation / coma / lethargia / desorientation
- Severe allergic reaction / atopy
- Venous thrombosis / phlebitis / thrombophlebitis

Occasional
- 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
- Lung / pulmonary infiltrates
- Lymphadenopathy / polyadenopathies
- Mitral valve prolapse / incompetence / insufficiency / regurgitation / ring anomaly
- Myositis
- Neuritis / polyneuritis / multineuritis
- Osteonecrosis / bone infarction
- 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 glomerular defect / glomerulopathy
- Retinopathy
- Retrobulbar optic neuritis
- Seizures / epilepsy / absences / spasms / status epilepticus
- Splenomegaly
- Transient cerebral ischemia / stroke
- Troubles of memory / amnesia / hypermnesia
- Xerophthalmia / dry eyes


Very frequent
- Erythema / erythematous lesions / erythroderma / polymorphous erythema
- Macules
- Positive Nikolski's sign / achantolysis

Frequent
- Feeding disorder / dysphagia / swallowing / sucking disorder / esophageal dyskinesia
- Hypersialorrhea
- Polynuclear cells / neutrophils anomalies / neutropenia

Occasional
- Abnormal hepatic enzymes / transaminases
- Acute hepatic failure
- Anaemia
- Chronic uveitis / blepharitis / episcleritis / scleritis / conjonctivitis / keratitis
- Collapse / sudden death / cardiac arrest / cardiorespiratory arrest
- Corneal ulceration / perforation
- Cough
- Dyspareunia / coital pain / vaginal dryness
- Ectropion / entropion / eyelid eversion
- Gastroesophageal reflux / pyrosis / esophagitis / hiatal hernia / gastroparesia
- Interstitial / restrictive pneumopathy / restrictive respiratory syndrome
- Phimosis / preputial adhesions / paraphimosis
- Pollakiuria / polyuria / dysuria / anuria / acute urine retention / oliguria
- Repeat respiratory infections
- Respiratory distress / dyspnea / respiratory failure / lung volume reduction
- Sepsis severe / septicemia
- Thirst
- Thrombocytopenia / thrombopenia
- Urethral anomalies / stenosis / posterior urethral valves / megalocystis