eMedicine Specialties > Dermatology > Reactive & Inflammatory Dermatoses

Behcet Disease: Differential Diagnoses & Workup

Author: Marjan Yousefi, MD, Department of Dermatology, Geisinger Medical Center
Coauthor(s): Tammie Ferringer, MD, Teaching Staff, Departments of Dermatology and Pathology, Geisinger Medical Center; Sungnack Lee, MD, Vice President of Medical Affairs, Professor, Department of Dermatology, Ajou University School of Medicine, Korea; Dongsik Bang, MD, PhD, Vice Director, Severance Hospital; Professor, Department of Dermatology, Yonsei University College of Medicine, Korea
Contributor Information and Disclosures

Updated: Jun 19, 2009

Differential Diagnoses

Acute Febrile Neutrophilic Dermatosis
Herpes Simplex
Aphthous Stomatitis
Lupus Erythematosus, Acute
Erythema Multiforme
Pyoderma Gangrenosum
Erythema Nodosum
Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis

Other Problems to Be Considered

Inflammatory bowel disease (Crohn disease)

Workup

Laboratory Studies

  • Behçet disease cannot be confirmed through clinical laboratory results.
    • Mild anemia and leukocytosis are observed in some patients with chronic disease.
    • The erythrocyte sedimentation rate, C-reactive protein value, and other acute phase reactants may be elevated during the active stage of Behçet disease, but they do not correlate well with the clinical activity.
    • An increase in alpha-2 globulins is often observed. Serum immunoglobulin levels, especially immunoglobulin A, may be elevated.
    • Circulating immune complexes are often present.
    • Rheumatoid factor and antinuclear antibodies are absent.
    • Antineutrophil cytoplasmic antibody and antiphospholipid antibody test results are usually negative.

Histologic Findings

The etiology and pathogenesis of Behçet disease remain obscure, although many reviews describe a lymphocytic vasculitis.

Vasculitis is thought to affect vessels of all sizes; the various skin lesions are thought to be secondary to small vessel vasculitis.

The histopathology is variable, dependent upon the type of lesion. Pathergic lesions are characterized by a heavy neutrophilic infiltrate without fibrin within the vessel walls. Folliculitis, acneiform lesions, and dermal abscesses have been described in Behçet disease.

The erythema nodosum–like lesions show a perivascular lymphocytic infiltrate of lymphocytes in the deep dermis and septa with a lymphocytic vasculitis but lack the histiocytic granulomas of typical erythema nodosum.

The aphthous ulcers have a nonspecific pathology with a variable infiltrate of lymphocytes, macrophages, and neutrophils at the base of the ulcer.

T-cell subsets with a preponderance of helper-inducer cells over T suppressor-cytotoxic cells have been observed in lesions.

Electron microscopic observations

Examination of erythema nodosum–like lesions shows microvascular changes and lymphocyte-mediated fat cell lysis. Additionally, small dermal blood vessels embolized by thrombi are observed at the sites of the needle prick reaction (pathergy) and at the erythema nodosum–like lesions.

The early changes in fat cells may be caused by vascular changes brought about by the specific degeneration of endothelial cells and vascular stenosis associated with the delayed-type hypersensitivity reaction.

More on Behcet Disease

Overview: Behcet Disease
Differential Diagnoses & Workup: Behcet Disease
Treatment & Medication: Behcet Disease
Follow-up: Behcet Disease
Multimedia: Behcet Disease
References
Further Reading
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Keywords

Behcet disease, Behcet's disease, Behçet disease, Behcet's disease, Behcet syndrome, Behcet's syndrome, oral aphthous ulcer, genital ulcer, uveitis, oral aphthae, oral ulcers, canker sores

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Contributor Information and Disclosures

Author

Marjan Yousefi, MD, Department of Dermatology, Geisinger Medical Center
Marjan Yousefi, MD is a member of the following medical societies: American Academy of Dermatology and Phi Beta Kappa
Disclosure: Nothing to disclose.

Coauthor(s)

Tammie Ferringer, MD, Teaching Staff, Departments of Dermatology and Pathology, Geisinger Medical Center
Tammie Ferringer, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, and American Society of Dermatopathology
Disclosure: Nothing to disclose.

Sungnack Lee, MD, Vice President of Medical Affairs, Professor, Department of Dermatology, Ajou University School of Medicine, Korea
Sungnack Lee, MD is a member of the following medical societies: American Dermatological Association
Disclosure: Nothing to disclose.

Dongsik Bang, MD, PhD, Vice Director, Severance Hospital; Professor, Department of Dermatology, Yonsei University College of Medicine, Korea
Disclosure: Nothing to disclose.

Medical Editor

David P Fivenson, MD, Associate Director, St Joseph Mercy Hospital Dermatology Program, Ann Arbor, Michigan
David P Fivenson, MD is a member of the following medical societies: American Academy of Dermatology, Medical Dermatology Society, Michigan Dermatological Society, Michigan State Medical Society, Photomedicine Society, Society for Investigative Dermatology, and Wound Healing Society
Disclosure: Nothing to disclose.

Pharmacy Editor

David F Butler, MD, Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic
David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Managing Editor

Warren R Heymann, MD, Head, Division of Dermatology, Professor, Department of Internal Medicine, University of Medicine and Dentistry of New Jersey
Warren R Heymann, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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