BEHCETS DISEASE

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BEHCETS DISEASE
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• Rare multisystem, chronic disease characterized
  by oral and genital mucocutaneous ulcerations,
  skin rashes, arthritis, thrombophlebitis,
  uveitis, colitis, and neurologic symptoms.
• Endemic in Japan and Northeastern Mediterranean
  region(Turkey Iran)

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EPIDEMIOLOGY

• Incidence/Prevalence in USA
• 1/100, 000 prevalence
• 670/100,000 in Japan
• Genetics One report in a mother and newborn (A.
  Fam, Ann Rheumatic Dis 1981 40509-512). Very
  rarely familial.

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EPIDEMIOLOGY

• Predominant age 3rd to 4th decades
• Predominant sex Male Female as frequently to
  twice as often. Some studies suggest equal
  frequency.

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SIGNS SYMPTOMS

• Aphthous stomatitis
• Genital ulcers - painful in the male, painless in
  the female
• Dermal - papulovesicular, erythema nodosum,
  pathergy, erythema multiforme, vasculitis,
  pyoderma
• Ocular - iritis, iridocyclitis, chorioretinitis,
  hypopyon, hemorrhage, papilledema, optic atrophy

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SIGNS SYMPTOMS

• Morning stiffness - in 1/3
• Polyarthritis - self-limited and predominantly
  affecting lower extremities
• Thrombophlebitis - peripheral, pulmonary,
  cerebral, Budd Chiari syndrome
• Neurologic - cranial nerve palsy, hemiplegia,
  intracranial hypertension, meningomyelitis and
  recurrent meningitis, confusional state

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SIGNS SYMPTOMS

• Gl - aphthous ulcers, colitis, melena
• Pulmonary infiltrates - possibly related to
  thrombosis
• Myopathy/myositis - rare
• Peripheral gangrene - rare
• Epididymitis
• Glomerulonephritis - rare

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HYPOPYON pus in the anterior chamber
associated with uveitis
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Diagnostic criteria

• Recurrent oral ulcers at least 3 times in a year
• Recurrent genital ulcers
• Eye inflammation
• Skin inflammation
• Pseudo folliculitis
• Acne-like lesions
• Erythema nodosum-like

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DIFFFERENTIAL

• Reiter's syndrome and other forms of
  spondyloarthropathy
• Inflammatory bowel disease (Crohn's disease and
  ulcerative colitis)
• Syphilis
• Erythema nodosum
• Aphthous stomatitis

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DIFFFERENTIAL

• Herpes simplex
• Stevens-Johnson syndrome
• Vasculitis
• Multisystem disease
• Thrombophlebitis related to coagulation factor
  deficiency

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LABS

• Erythrocyte sedimentation rate elevation, but can
  be normal
• Immune complexes detected by Raji cell and C1q
  solid phase assays
• Cryoglobulin
• Hypergammaglobulinemia
• Circulating anticoagulation (rare)
• Anti-cardiolipin antibody (rare)

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Special Tests

• None specific for Behcet's, but helpful in
  following disease course
• Depression of plasma antithrombin III levels with
  active disease
• Increased fibrinolytic activity during attacks
• Anti-neutrophil cytoplasmic antigen antibodies,
  perinuclear variety

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Special Tests

• Demyelinating antibodies in neuro-Behcet's
  syndrome
• Anti-cardiolipin antibodies, lupus anticoagulants
  
• Anti-endothelial antibodies

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TREATMENT

• Colchicine 0.6 mg bid
• Topical ocular steroids
• Prednisone 1 mg/kg for severe involvement,
  especially CNS
• Azathioprine 2-3 mg/kg/day po
• Methotrexate Use the lowest possible dose
  perhaps 7.5 mg/week

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Others

• Cyclosporine
• Levamisole - 100-150 mg two days per week
• Chlorambucil - but concern with respect to
  toxicity, especially its malignant potential
• Thalidomide
• Cyclophosphamide 50-100 mg/day q am. Patient
  should drink 8-10 glasses of water/day and report
  any blood in the urine.

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Complications

• Death
• Blindness
• Paralysis
• Embolism/thrombosis - pulmonary, vena cava,
  peripheral
• Aneurysms
• Amyloidosis
• Thrombotic events, especially when
  anticardiolipin antibodies present

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• Normal life expectancy, except with neurologic
  involvement
• Possible vision impairment
• Avoid English Walnuts!!

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SWEET SYNDROME

• Presents the sameno vascular damage!
• Acute febrile neutrophilic dermatosis

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