eMedicine Specialties > Pediatrics: General Medicine > Rheumatology

Behcet Syndrome: Differential Diagnoses & Workup

Author: C Egla Rabinovich, MD, MPH,, Assistant Professor and Co-Division Director, Department of Pediatrics, Division of Pediatric Rheumatology, Duke University Medical Center
Coauthor(s): Linda Wagner-Weiner, MD, Assistant Professor, Department of Pediatrics, University of Chicago
Contributor Information and Disclosures

Updated: Aug 24, 2009

Differential Diagnoses

Adrenal Insufficiency
Systemic Lupus Erythematosus
Arthritis, Conjunctivitis, Urethritis Syndrome
Ulcerative Colitis
Crohn Disease
Herpes Simplex Virus Infection
Sarcoidosis

Other Problems to Be Considered

Primary antiphospholipid antibody syndrome
Multiple sclerosis
Stevens-Johnson syndrome
Familial Mediterranean fever
Syndrome of periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA)

Workup

Laboratory Studies

  • No specific laboratory test result is diagnostic of Behçet syndrome.
    • Serum complement levels are within the reference range, except for just prior to eye or mucous membrane involvement, at which time they may be decreased.
    • Sedimentation rate or C-reactive protein may be elevated. Chronic anemia common, and a neutrophil leukocytosis is seen in about 15% of patients.
    • Human leukocyte antigen (HLA)-B51 may be present in patients of Asian, Mexican, or Middle Eastern descent.
    • Anticardiolipin antibodies are present in as many as 30% of patients.
  • Systemic lupus erythematosus and other vasculitic syndromes must be ruled out. Patients with Behcet syndrome have negative antinuclear and antineutrophilic cytoplasmic antibodies.
  • In patients with CNS findings, cerebral spinal fluid pleocytosis may be present.
  • In addition to thrombosis associated with antiphospholipid antibodies, thrombosis has been reported in Behçet syndrome associated with factor V Leiden mutations and with prothrombin G20210A mutations.

Imaging Studies

  • Brain MRI and/or CT scanning for visualization of the neurological lesions is often helpful in patients with CNS involvement. Focal lesions may be observed anywhere in the CNS on the MRI, appearing as high signal on the T2-weighted images and low signal on the T1-weighted images. Flare images may be especially helpful. Enlargement of ventricles or subarachnoid spaces may be observed. However, the MRI findings of the brain may be normal even in the presence of neurologic involvement. Neuropsychologic testing results may be abnormal prior to any detectable lesions on neuro-imaging.
  • Angiography shows areas of aneurysm formation and thrombosis.
  • Echocardiography is useful in patients with murmurs because it is useful for diagnosing the valve vegetations and ventricular thrombi, which can occur in Behçet syndrome.
    MRI, T2-weighted images of brainstem involvement ...

    MRI, T2-weighted images of brainstem involvement with meningoencephalitis in an 11-year-old girl with neurologic Behçet syndrome.

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    MRI, T2-weighted images of brainstem involvement ...

    MRI, T2-weighted images of brainstem involvement with meningoencephalitis in an 11-year-old girl with neurologic Behçet syndrome.

Other Tests

  • Endoscopy of the GI tract is useful for detecting gastrointestinal ulcerations.
  • A thorough eye examination by an experienced ophthalmologist is essential. Consider fluorescein angiography for evaluation of retinal vessels. Follow-up visits with an ophthalmologist should be scheduled at least every 6-12 months.
  • Neuropsychologic testing may be useful with CNS involvement, revealing memory impairment or personality changes, and can be useful in monitoring neuropsychologic status.

Histologic Findings

  • Behçet syndrome is diagnosed clinically, not by means of tissue evaluation. However, round cell infiltration may be found in cardiac valve lesions.
  • Biopsy of the buccal and genital ulcers reveals lymphocytic and plasma cell invasion in the prickle cell layer of the epidermis.
  • Dermal vessels are infiltrated with lymphocytes and plasma cells with immune deposits of immunoglobulin M (IgM) and C3. Occasionally, necrotizing vasculitis is observed.

    Histology of ulcers revealing neutrophilic infilt...

    Histology of ulcers revealing neutrophilic infiltrate and vasculitis.

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    Histology of ulcers revealing neutrophilic infilt...

    Histology of ulcers revealing neutrophilic infiltrate and vasculitis.

More on Behcet Syndrome

Overview: Behcet Syndrome
Differential Diagnoses & Workup: Behcet Syndrome
Treatment & Medication: Behcet Syndrome
Follow-up: Behcet Syndrome
Multimedia: Behcet Syndrome
References
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References

  1. Al-Araji A, Kidd DP. Neuro-Behcet's disease: epidemiology, clinical characteristics, and management. Lancet Neurol. Feb 2009;8(2):192-204. [Medline].

  2. Ebert EC. Gastrointestinal manifestations of Behçet's disease. Dig Dis Sci. Feb 2009;54(2):201-7. [Medline].

  3. Ihle J, Kummerle-Deschner J, Orlikowsky T, Albert E, Niethammer D, Dannecker GE. Factor V Leiden and venous thrombosis in a 4-yr-old girl with Behcet's syndrome. Rheumatology (Oxford). Feb 2000;39(2):209-10. [Medline].

  4. [Guideline] Hatemi G, Silman A, Bang D, Bodaghi B, Chamberlain AM, Gul A. EULAR recommendations for the management of Behçet disease. Ann Rheum Dis. Dec 2008;67(12):1656-62. [Medline].

  5. Alpsoy E. Behcet's disease: treatment of mucocutaneous lesions. Clin Exp Rheumatol. Jul-Aug 2005;23(4):532-9. [Medline].

  6. Fresko I, Soy M, Hamuryudan V, et al. Genetic anticipation in Behcet's syndrome. Ann Rheum Dis. Jan 1998;57(1):45-8. [Medline].

  7. Gerber S, Biondi A, Dormont D, Wechsler B, Marsault C. Long-term MR follow-up of cerebral lesions in neuro-Behcet's disease. Neuroradiology. Nov 1996;38(8):761-8. [Medline].

  8. Kesen MR, Goldstein DA, Tessler HH. Uveitis associated with pediatric Behcet disease in the american midwest. Am J Ophthalmol. Dec 2008;146(6):819-27.e2. [Medline].

  9. Kikuchi H, Aramaki K, Hirohata S. Effect of infliximab in progressive neuro-Behcet's syndrome. J Neurol Sci. Sep 15 2008;272(1-2):99-105. [Medline].

  10. Kone-Paut I, Geisler I, Wechsler B, et al. Familial aggregation in Behcet's disease: high frequency in siblings and parents of pediatric probands. J Pediatr. Jul 1999;135(1):89-93. [Medline].

  11. Marshall SE. Behcet's disease. Best Pract Res Clin Rheumatol. Jun 2004;18(3):291-311. [Medline].

  12. Martini A. Behcet's disease and Takayasu's disease in children. Curr Opin Rheumatol. Sep 1995;7(5):449-54. [Medline].

  13. [Best Evidence] Melikoglu M, Fresko I, Mat C, et al. Short-term trial of etanercept in Behçet's disease: a double blind, placebo controlled study. J Rheumatol. Jan 2005;32(1):98-105. [Medline].

  14. Nakamura S, Ohno S. Anti-tumor necrosis factor alpha antibody in the treatment of Behcet's disease. Int Ophthalmol Clin. 2005;45(2):179-89. [Medline].

  15. Oktem-Tanor O, Baykan-Kurt B, Gurvit IH, et al. Neuropsychological follow-up of 12 patients with neuro-Behcet disease. J Neurol. Feb 1999;246(2):113-9. [Medline].

  16. Rakavor Y, Adar H, Tal I, et al. Behcet Disease: Long-term follow-up of three children and review of the literature. Pediatrics. 1989;83:986-92. [Medline].

  17. Sakane T, Takeno M, Suzuki N, Inaba G. Behcet's disease. N Engl J Med. Oct 21 1999;341(17):1284-91. [Medline].

  18. Tabbara KF, Al-Hemidan AI. Infliximab effects compared to conventional therapy in the management of retinal vasculitis in Behçet disease. Am J Ophthalmol. Dec 2008;146(6):845-50.e1. [Medline].

  19. Yazici H, Pazarli H, Barnes CG, et al. A controlled trial of azathioprine in Behcet's syndrome. N Engl J Med. Feb 1 1990;322(5):281-5. [Medline].

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Further Reading

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Keywords

Behçet syndrome, Behçet's disease, Behçet disease, Behcet syndrome, Behcet's syndrome, Adamantiades-Behçet's disease, Adamantiades-Behçet disease, blindness, aphthous ulcers, genital ulcers, vasculitis, Budd-Chiari syndrome, eye lesions, skin lesions, erythema nodosum, pseudofolliculitis, uveitis, cutaneous pustular vasculitis, synovitis, meningoencephalitis, treatment, diagnosis 

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Contributor Information and Disclosures

Author

C Egla Rabinovich, MD, MPH,, Assistant Professor and Co-Division Director, Department of Pediatrics, Division of Pediatric Rheumatology, Duke University Medical Center
C Egla Rabinovich, MD, MPH, is a member of the following medical societies: American College of Rheumatology
Disclosure: Nothing to disclose.

Coauthor(s)

Linda Wagner-Weiner, MD, Assistant Professor, Department of Pediatrics, University of Chicago
Linda Wagner-Weiner, MD is a member of the following medical societies: American Academy of Pediatrics and American College of Rheumatology
Disclosure: Nothing to disclose.

Pharmacy Editor

Mary L Windle, PharmD, Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy, Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Thomas JA Lehman, MD, FAAP, FACR, Clinical Professor of Pediatrics, Department of Pediatrics, Division of Pediatric Rheumatology, Weill-Cornell University; Chief, Hospital for Special Surgery
Thomas JA Lehman, MD, FAAP, FACR is a member of the following medical societies: PM American Allergy Society
Disclosure: Nothing to disclose.

CME Editor

Paul D Petry, DO, FACOP, FAAP, Consulting Staff, Freeman Pediatric Care, Freeman Health System
Paul D Petry, DO, FACOP, FAAP is a member of the following medical societies: American Academy of Osteopathy, American Academy of Pediatrics, American College of Osteopathic Pediatricians, and American Osteopathic Association
Disclosure: Nothing to disclose.

Chief Editor

Lawrence K Jung, MD, Chief, Division of Pediatric Rheumatology, Children's National Medical Center
Lawrence K Jung, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Rheumatology, Clinical Immunology Society, and New York Academy of Sciences
Disclosure: Nothing to disclose.

 
 
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