Rheumatology Book

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Behcet's Syndrome

Aka: Behcet's Syndrome, Behcet's Disease, Pathergy Test
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  1. See Also
    1. Aphthous Ulcer
    2. Genital Ulcer
    3. Vasculitis
  2. Background
    1. Pronunciation: "Buh shettes"
    2. First described by Hippocrates in fifth century B.C.
    3. Turkish Dermatologist Hulusi Behcet described 1937
  3. 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
  4. Pathophysiology
    1. Autoimmune Vasculitis
    2. HLA-B51/B5 Allele (found in 57% of Behcet Syndrome patients)
  5. Symptoms
    1. Morning stiffness
    2. Oral and Genital Ulcers
    3. Skin rashes
    4. Joint pain
    5. Neurologic changes
  6. 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
  7. 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
  8. 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. Prick Forearm with small, sterile needle
      2. Positive if small red Pustule (2 mm) forms at site within 24-48 hours
    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
  9. 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
  10. 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
  11. 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
  12. Course
    1. Spontaneous regression without treatment may occur
  13. References
    1. Ghate in Ruddy (2001) Kelley's Rheumatology, p. 1205-8
    2. Ghate (1999) J Am Acad Dermatol 40(1):1-18
    3. Roett (2012) Am Fam Physician 85(3): 254-62

Behcet Syndrome (C0004943)

Definition (NCI) A rare chronic inflammatory disorder of unknown etiology. It is characterized by the development of ulcers in the mouth and genital region and uveitis. Other signs and symptoms include arthritis, deep vein thrombosis and superficial thrombophlebitis.
Definition (MEDLINEPLUS)

Behcet's syndrome is a disease that involves inflammation of the blood vessels. It causes problems in many parts of the body. The most common symptoms are

  • Sores in the mouth
  • Sores on the sex organs
  • Other skin sores
  • Swelling of parts of the eye
  • Pain, swelling and stiffness of the joints

More serious problems can include meningitis, blood clots, inflammation of the digestive system and blindness.

Doctors aren't sure what causes Behcet's. It is rare in the United States, but is common in the Middle East and Asia. It mainly affects people in their 20s and 30s. Diagnosing Behcet's can take a long time, because symptoms may come and go, and it may take months or even years to have all of the symptoms. There is no cure. Treatment focuses on reducing pain and preventing serious problems.

NIH: National Institute of Arthritis and Musculoskeletal and Skin Diseases
Definition (CSP) rare chronic inflammatory disease involving the small blood vessels; of unknown etiology and characterized by mucocutaneous ulceration in the mouth and genital region and uveitis with hypopyon; the neuroocular form may cause blindness and death; synovitis, thrombophlebitis, gastrointestinal ulcerations, retinal vasculitis, and optic atrophy may occur as well.
Definition (MSH) Rare chronic inflammatory disease involving the small blood vessels. It is of unknown etiology and characterized by mucocutaneous ulceration in the mouth and genital region and uveitis with hypopyon. The neuro-ocular form may cause blindness and death. SYNOVITIS; THROMBOPHLEBITIS; gastrointestinal ulcerations; RETINAL VASCULITIS; and OPTIC ATROPHY may occur as well.
Concepts Disease or Syndrome (T047)
MSH D001528
ICD9 136.1
ICD10 M35.2
SnomedCT 41225007, 154424000, 310701003
English Behcet's Syndrome, Behcets Syndrome, Triple Symptom Complex, Triple-Symptom Complex, ADAMANTIADES-BEHCET SYNDROME, BEHCET RECURRENT DISEASE, BEHCET TRIPLE SYMPTOM COMPLEX, Behcet's Disease, OCULOBUCCOGENITAL SYNDROME, Behcet's, Behcet's disease, NOS, Behcet's syndrome, NOS, BEHCET SYNDROME, BD, BEHCET DISEASE, Behcet Syndrome, BEHCET DIS, Behcet's syndrome (diagnosis), Behcet's disease (disorder), Behcets disease, Behcet syndrome, Behçet's disease, Behcet Syndrome [Disease/Finding], behcet syndrome, behcet's syndrome, behcet's disease, behcets syndrome, behcet disease, behcet diseases, behcet's, behcets disease, syndrome behcet's, Behcet Disease, triple symptom complex, Behcet's disease, Behcet's syndrome, Behcet's syndrome (disorder), Behcet, Behçet, Beh?et's syndrome (disorder), Beh?et's syndrome, Behçet's syndrome (disorder), Behçet's syndrome, Adamantiades-Behcet disease
Italian Sindrome di Bechet, Malattia di Behcet, Sindrome di Behcet
Dutch ziekte van Behçet, Ziekte van Behet, syndroom van Behçet, Tripelsymptomencomplex, Oculo-genitaal syndroom, Oculo-urogenitaal syndroom, Ziekte van Behçet
French Maladie de Behcet, Syndrome de Behcet, Maladie de Behçet, Syndrome oculo-muco-cutané de Behçet, Grande aphtose de Touraine, Syndrome d'Adamantiades-Behçet, Syndrome de Behçet
German Behcet-Syndrom, Behcet-Krankheit, Behcet Krankheit, Trisymptomkomplex, Behçet-Krankheit, Behçet-Syndrom, Morbus Behçet
Swedish Behcets syndrom
Japanese ﾍﾞｰﾁｪｯﾄﾋﾞｮｳ, ﾍﾞｰﾁｪｯﾄｼｮｳｺｳｸﾞﾝ, Behcet症候群, ベーチェット病, ベーチェット症候群, 三症状複合体, Behcet病
Czech Behcetův syndrom, Behcetova nemoc, Behcetova choroba
Spanish Behcet's disease, enfermedad de Behçet, enfermedad de Behcet, síndrome de Behcet (concepto no activo), síndrome de Behcet (trastorno), síndrome de Behcet, Sindrome de Behcet, Complejo de Sintoma Triple, Enfermedad de Behcet, Complejo de Síntoma Triple, Enfermedad de Behçet, Síndrome de Behçet
Finnish Behçetin oireyhtymä
Russian BEKHCHETA SINDROM, TRIADY SIMPTOMOV KOMPLEKS, БЕХЧЕТА СИНДРОМ, ТРИАДЫ СИМПТОМОВ КОМПЛЕКС
Korean 베제트 병
Polish Zespół Behceta, Choroba Behceta, Zespół Hulusi-Behceta
Hungarian Behçet syndroma, Behçet-betegség, Behçet-syndroma, Behçet betegség
Portuguese Complexo de Triplo Sintoma, Doença de Behçet, Síndrome de Behçet
Sources
Derived from the NIH UMLS (Unified Medical Language System)


pathergy test (C3161802)

Concepts Laboratory Procedure (T059)
English pathergy test, pathergy test (lab test)
Sources
Derived from the NIH UMLS (Unified Medical Language System)


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