Rheumatology Book

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Behcet's SyndromeAka: Behcet's Disease, Pathergy Test

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  1. Background
    1. Pronunciation: "Buh shettes"
    2. First described by Hippocrates in fifth century B.C.
    3. Turkish Dermatologist Hulusi Behcet described 1937
  2. Epidemiology
    1. Endemic in Japan, Middle East, Greece, Cyprus, Turkey
    2. Incidence
      1. United States: 1:100,000
      2. Japan: 670:100,000
    3. Ages: 25-50 years
    4. Sex: Males more often affected than women
  3. Pathophysiology
    1. Autoimmune Vasculitis
    2. HLA-B51 relationship
  4. Symptoms
    1. Morning stiffness
    2. Oral and Genital Ulcers
    3. Skin rashes
    4. Joint pain
    5. Neurologic changes
  5. Signs
    1. Aphthous stomatitis
    2. Skin
      1. Papulovesicular, Vasculitis
      2. Erythema Nodosum
      3. Erythema Multiforme
    3. Ocular
      1. Iritis
      2. Iridocyclitis
      3. Chorioretinitis
      4. Hypopyon
      5. Papilledema
      6. Optic Nerve atrophy
    4. Thrombophlebitis
    5. Neurologic
      1. Intracranial Hypertension
      2. Cranial Nerve palsy
      3. Meningitis
    6. Polyarthritis
  6. Differential Diagnosis
    1. Crohn's Disease
    2. HyperEosinophilic Syndrome
    3. Pemphigoid
    4. Lichen Planus
    5. Reiter's Syndrome
    6. Ulcerative Colitis
    7. Syphilis
    8. Erythema Nodosum
    9. Aphthous stomatitis
    10. Steven's Johnson syndrome
    11. Herpes Simplex Stomatitis
    12. Lyme Disease
  7. Labs
    1. Erythrocyte Sedimentation Rate elevated
    2. Cryoglobulin
    3. Hypergammaglobulinemia
    4. Biopsy
      1. Leukocytoclastic Vasculitis
    5. Pathergy Test (not sensitive, relatively specific)
      1. Prick forearm with small, sterile needle
      2. Positive if small red Pustule forms at site
  8. Management
    1. Colchicine 0.6 mg bid
    2. Topical ocular steroid
    3. Prednisone 1 mg/kg for severe cases
    4. Azathioprine 2-3 mg/kg/day PO qd
    5. Cyclophosphamide 50-100 mg/day qAM
      1. Take with 64 ounces/day fluid
      2. Observe for Hematuria
    6. Methotrexate 7.5 mg/week or lowest effective dose
    7. Experimental therapies
      1. Thalidomide
      2. Interferon-alpha
  9. References
    1. Ghate in Ruddy (2001) Kelley's Rheumatology, p. 1205-8
    2. Ghate (1999) J Am Acad Dermatol 40(1):1

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