Behcet's Syndrome

Aka: Behcet's Syndrome, Behcet's Disease, Behcet Syndrome, Adamantiades-Behcet Syndrome, Behðcet's Disease, Behcet Triple Sydrome Complex

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II. Background

  1. Pronunciation: "Buh shettes"
  2. First described by Hippocrates in fifth century B.C.
  3. Turkish Dermatologist Hulusi Behcet described 1937

III. Epidemiology

  1. Endemic regions
    1. Japan
    2. Eastern Mediterranean (Middle East, Greece, Cyprus, Turkey)
  2. Incidence
    1. United States: 1:100,000
    2. Japan: 670:100,000
  3. Ages: 25-50 years
  4. Gender
    1. Males predominate in Japan
    2. Women predominate in United States

IV. Pathophysiology

  1. Autoimmune Vasculitis
  2. HLA-B51/B5 Allele (found in 57% of Behcet Syndrome patients)

V. Symptoms

  1. Morning stiffness
  2. Oral and Genital Ulcers
  3. Skin rashes
  4. Joint Pain
  5. Neurologic changes

VI. Signs

  1. Mucocutaneous ulcers
    1. Aphthous Stomatitis (100% of cases)
    2. Genital Ulcers (70-90% of cases)
  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

VII. 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

VIII. Labs: General

  1. Antibodies to SIP1 Carboxy-terminal subunit may be positive
  2. Biopsy
    1. Leukocytoclastic Vasculitis diffusely involving the arterys and veins
  3. Pathergy Test (not sensitive, relatively specific)
    1. See Pathergy Test
    2. Pustule or ulceration forms within 24-48 hours in response to minor Skin Trauma (e.g. SQ Injection)
  4. Non-specific findings
    1. Erythrocyte Sedimentation Rate elevated
    2. Cryoglobulin
    3. Hypergammaglobulinemia
  5. Other rheumatologic labs to consider
    1. Antinuclear Antibody (ANA)
    2. Rheumatoid Factor

IX. Diagnosis: Criteria

  1. Recurrent Aphthous Ulcers (more than 3 per year) and
  2. Two or more of the following criteria
    1. Recurrent Genital Ulcers
    2. Uveitis or other eye lesions
    3. Erythema Nodosum or other skin lesions
    4. Pathergy Test positive (see above)
    5. Biopsy demonstrating diffuse arteritis and venulitis

X. Management: First-Line treatment of mucocutaneous lesions

  1. Pegylated Interferon Alfa-2A (Pegasys)
    1. Dose: 6 Million units subcutaneously three times weekly for 3 months
    2. Alpsoy (2002) Arch Dermatol 138(4): 467-71 [PubMed]

XI. Management: Other treatments that have been used for systemic manifestations

  1. Aphthous Ulcers
    1. Topical Sucralfate
  2. Uveitis
    1. Topical ocular steroid
  3. Systemic agents
    1. Colchicine 0.6 mg bid
    2. Prednisone 1 mg/kg for severe cases
    3. Azathioprine 2-3 mg/kg/day PO qd
    4. Cyclophosphamide 50-100 mg/day qAM
      1. Take with 64 ounces/day fluid
      2. Observe for Hematuria
    5. Methotrexate 7.5 mg/week or lowest effective dose

XII. Course

  1. Spontaneous regression without treatment may occur

XIII. References

  1. Ghate in Ruddy (2001) Kelley's Rheumatology, p. 1205-8
  2. Ghate (1999) J Am Acad Dermatol 40(1):1-18 [PubMed]
  3. Roett (2012) Am Fam Physician 85(3): 254-62 [PubMed]

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