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Dermatologic Aspects of Behcet Disease

  • Author: Amira M Elbendary, MBBCh, MSc; Chief Editor: William D James, MD  more...
 
Updated: Jan 12, 2016
 

Background

Behçet disease (BD) was named in 1937 after the Turkish dermatologist Hulusi Behçet, who described the triple-symptom complex of recurrent oral aphthous ulcers, genital ulcers, and uveitis ,[1] but it is likely that Behçet disease was already described years earlier by Hippocrates, who described a disease with aphthous ulcers and defluxion around genital area, associated with warty ophthalmic conditions.[2]

Behçet disease is a complex, multisystemic disease that includes involvement of the mucocutaneous, ocular, cardiovascular, renal, gastrointestinal, pulmonary, urologic, and central nervous systems, as well as the joints, blood vessels, and lungs.

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Pathophysiology

The cause of Behçet disease is not known; however, immunogenetics, immune regulation, vascular abnormalities (including arterial and venous abnormalities, affecting small, medium, and large vessels), or bacterial and viral infection may have a role in its development.

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Epidemiology

Frequency

United States

Behçet disease is not common in the United States, with a prevalence of 5 cases per 100,000 persons.

International

A wide geographic variation is noted in the frequency of Behçet disease, with high prevalence in Mediterranean area compared with the United States and Northern Europe. Studies have shown that immigrants from areas with high prevalence rate of Behçet disease remain at high risk of developing Behçet disease.[3] Behçet disease is most prevalent (and more virulent) in the Mediterranean region, Middle East, and Far East, with an estimated prevalence of 1 case per 10,000 persons.

The prevalence reported in different countries is as follows[3] :

  • Turkey: 20-420 cases per 100,000 population
  • Iran: 16-68 cases per 100,000 population
  • Israel: 15-120 cases per 100,000 population
  • Iraq: 17 cases per 100,000 population
  • Egypt: 7.6 cases per 100,000 population
  • Spain: 7.5 cases per 100,000 population
  • Japan: 7-13.5 cases per 100,000 population
  • Martinique: 7 cases per 100,000 population
  • France: 7.1 cases per 100,000 population
  • Italy: 3.8-16 cases per 100,000 population
  • Northern Europe: 0.5-5 cases per 100,000 population

Sex

Men are affected more often, and with more severe disease, than women in some Mediterranean areas. In Iran, for example, the male-to-female ratio was 24:1 among 1712 patients. In Turkey, the ratio was 16:1 among 427 patients.

Age

Onset can occur at any age, but is it most common during the third decade of life.[4] Behçet disease rarely occurs in individuals older than 50 years or during childhood.[5] Genetic anticipation was reported in Behçet disease, in which the onset of the disease becomes earlier with successive generations.[6]

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Mortality/Morbidity

Chronic morbidity is typical; the leading cause is ophthalmic involvement, which can result in blindness. The effects of the disease may be cumulative, especially with neurologic, vascular, and ocular involvement.

The mortality rate is low, but death can occur from neurologic involvement, vascular disease, bowel perforation, cardiopulmonary disease, or as a complication of immunosuppressive therapy.[7]

A recent study has shown that sub-Saharan African patients with Behçet disease had three times higher mortality compared with North African and European patients, with the 15-year mortality rate reaching 20%, compared with mortality rates of about 5-10% reported in other countries.[8]

Clinical expression of the disease also shows geographic variation. Sub-Saharan African patients show higher CNS and cardiovascular involvement compared with Behçet disease patients in North Africa and Europe.

Gastrointestinal manifestations and neurological involvement was found to occur at higher rates in American patients compared with Turkish patients.[8]

Higher rates of folliculitis were found in patients of Iraqi/Iranian origin, and ocular complications were found to be at higher incidence in patients of Arabic origin in a study done on 66 Jewish patients.[8]

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Prognosis

The clinical course of Behçet disease is variable, even in the early stages, making determinations of the long-term prognosis difficult.

Men appear to have a poorer prognosis.

The disease usually runs a protracted course, with attacks generally lasting for several weeks and recurring more frequently early in the disease.

Mucocutaneous and arthritic involvement usually occur early.

Chronic morbidity is usual; the leading cause is ophthalmic involvement, which can result in blindness. The effects of the disease may be cumulative, especially for neurologic, vascular, and ocular involvement.

Mortality is low, but patients may die from neurologic involvement, vascular disease, bowel perforation, cardiopulmonary disease, or as a complication of immunosuppressive therapy.

The risk of cancer was evaluated in a large cohort study including 1,314 patients with Behçet disease. Non-Hodgkin lymphoma, hematologic malignancies, and female breast cancer were the most frequent malignancies observed. The cancer risk was found to be highest within the first year of follow up.[9]

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Patient Education

For patient education resources, see the Teeth and Mouth Center, as well as Canker Sores.

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Contributor Information and Disclosures
Author

Amira M Elbendary, MBBCh, MSc Visiting Research Fellow, Ackerman Academy of Dermatopathology; Teaching Assistant, Department of Dermatology, Kasr Alainy University Hospital, Cairo University, Egypt

Amira M Elbendary, MBBCh, MSc is a member of the following medical societies: Medical Dermatology Society, Bloom’s Syndrome Association, Egyptian Medical Syndicate, International Dermoscopy Society

Disclosure: Nothing to disclose.

Coauthor(s)

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: American Medical Association, Alpha Omega Alpha, Association of Military Dermatologists, American Academy of Dermatology, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Phi Beta Kappa

Disclosure: Nothing to disclose.

Warren R Heymann, MD Head, Division of Dermatology, Professor, Department of Internal Medicine, Rutgers New Jersey Medical School

Warren R Heymann, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Chief Editor

William D James, MD Paul R Gross Professor of Dermatology, Vice-Chairman, Residency Program Director, Department of Dermatology, University of Pennsylvania School of Medicine

William D James, MD is a member of the following medical societies: American Academy of Dermatology, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

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, Michigan State Medical Society, Society for Investigative Dermatology, Photomedicine Society, Wound Healing Society, Michigan Dermatological Society, Medical Dermatology Society

Disclosure: Nothing to disclose.

Acknowledgements

Dongsik Bang, MD, PhD Vice Director, Severance Hospital; Professor, Department of Dermatology, Yonsei University College of Medicine, Korea

Disclosure: Nothing to disclose.

Tammie Ferringer, MD Dermatopathology Section Head, Dermatopathology Fellowship Director, 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, American Society of Dermatopathology, and International Society of Dermatopathology

Disclosure: Nothing to disclose.

Eun-So Lee, MD, PhD Chairman, Professor of Dermatology, Department of Dermatology, Ajou University School of Medicine, Korea

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.

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.

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Minor aphthous ulcer.
Major aphthous ulcer.
Herpetiform oral ulcer.
A characteristic genital ulcer on scrotum.
A single ulcer on vulva.
Erythema nodosum–like lesions on skin.
Papulopustular eruptions.
Sweet syndrome–like lesion.
Typical positive pathergy reaction at injection site.
Ocular involvement showing posterior uveitis.
CT angiogram showing pulmonary artery aneurysm in a Behçet disease patient. Courtesy of Mohanad Elfishawi, MD, Rheumatology Department, Cairo University.
Acneiform lesions in a Behçet disease patient. Courtesy of Mohanad Elfishawi, MD, Rheumatology Department, Cairo University.
Superficial thrombophlebitis in a Behçet disease patient. Courtesy of Mohanad Elfishawi, MD, Rheumatology Department, Cairo University.
Postphlebitic limb in a Behçet disease patient. Courtesy of Mohanad Elfishawi, MD, Rheumatology Department, Cairo University.
Acneiform lesions in a patient with Behçet disease (an inflamed nodule and noninflammatory comedones). Courtesy of Mohanad Elfishawi, MD, Rheumatology Department, Cairo University.
Radial artery aneurysm with its effect distally on index finger in a Behçet disease patient. Courtesy of Mohanad Elfishawi, MD, Rheumatology Department, Cairo University.
 
 
 
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