Sections

DDx

Diagnostic Considerations

Other conditions to consider are as follows:

Malignancy ^[46]
HLA-B27–associated syndromes ( ankylosing spondylitis, reactive arthritis, psoriatic arthritis)
Inflammatory bowel disease
HIV/AIDS
Hypercoagulable states (eg, protein C and S deficiency, factor V Leiden, hyperhomocysteinemia, prothrombin deficiency)
Alternative causes of uveitis
Viral and bacterial infections (eg, herpes simplex virus infection, chancroid)
Nutritional/hematological deficiencies
MAGIC syndrome (mouth and genital ulcers with inflamed cartilage syndrome)
Reactive arthritis
PFAPA syndrome (periodic fever, aphthous-stomatitis, pharyngitis, adenitis syndrome)
Sarcoidosis
Drug reactions

Neurologic Behçet disease must be considered in the differential diagnosis of a variety of neurologic syndromes, including the following:

Stroke in young adults
Multiple sclerosis
Movement disorders
Intracranial hypertension
Intracranial sinovenous occlusive diseases

Ophthalmologic considerations

It is important to consider other forms of uveitis in the differential diagnosis, especially in those patients who have a mild or atypical presentation of Behçet disease.

Human leukocyte antigen B27 (HLA-B27)–related anterior uveitis also may cause recurrent iridocyclitis with hypopyon, but it is typically unilateral.

Since Behçet disease is a bilateral panuveitis, other inflammatory processes that affect both eyes must be considered. Syphilis causes a retinitis with vitreitis rather than a strict vasculitis. The diagnosis for syphilis is confirmed by serology.

Sarcoidosis, another bilateral inflammatory process, may produce posterior pole findings similar to those in Behçet disease but is generally more indolent, in contrast to the explosive recurrent attacks of Behçet disease. Furthermore, the vasculitis seen in sarcoidosis usually is not occlusive in nature and typically involves only veins, compared with the involvement of both arteries and veins in Behçet disease.

Collagen vascular diseases and viral retinitis also may mimic the ocular changes of Behçet disease.

Differential Diagnoses

Workup

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Media Gallery

Oral aphthous ulcers secondary to Behçet disease.
Behçet disease. T2-weighted magnetic resonance images show brainstem involvement with meningoencephalitis in an 11-year-old girl with neurologic Behçet disease.
Histology of Behçet disease ulcers revealing neutrophilic infiltrate and vasculitis.

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Author

Nicole Davey-Ranasinghe, MD Rheumatologist, Allergy ARTS, LLP, Amarillo Center for Clinical Research, Ltd

Nicole Davey-Ranasinghe, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology

Disclosure: Nothing to disclose.

Coauthor(s)

Constantine K Saadeh, MD President, Allergy ARTS, LLP; Principal Investigator, Amarillo Center for Clinical Research, Ltd

Constantine K Saadeh, MD is a member of the following medical societies: American Academy of Allergy, Asthma and Immunology, American College of Rheumatology, American Medical Association, Southern Medical Association, Texas Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Lawrence H Brent, MD Associate Professor of Medicine, Sidney Kimmel Medical College of Thomas Jefferson University; Chair, Program Director, Department of Medicine, Division of Rheumatology, Albert Einstein Medical Center

Lawrence H Brent, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Physicians, American College of Rheumatology

Disclosure: Stock ownership for: Johnson & Johnson.

Chief Editor

Herbert S Diamond, MD Visiting Professor of Medicine, Division of Rheumatology, State University of New York Downstate Medical Center; Chairman Emeritus, Department of Internal Medicine, Western Pennsylvania Hospital

Herbert S Diamond, MD is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians, American College of Rheumatology, American Medical Association, Phi Beta Kappa

Disclosure: Nothing to disclose.

Additional Contributors

Jeffrey R Lisse, MD, FACP Professor, Department of Internal Medicine, Chief, Section of Rheumatology, University of Arizona School of Medicine

Jeffrey R Lisse, MD, FACP is a member of the following medical societies: Alpha Omega Alpha, American College of Physicians-American Society of Internal Medicine, American College of Rheumatology, American Geriatrics Society, Sigma Xi, The Scientific Research Honor Society

Disclosure: Received consulting fee from Genentech for consulting; Received consulting fee from Centacor for consulting; Received consulting fee from Novartis for review panel membership.

Kristine M Lohr, MD, MS Professor, Department of Internal Medicine, Interim Chief, Division of Rheumatology, Director, Rheumatology Training Program, University of Kentucky College of Medicine

Kristine M Lohr, MD, MS is a member of the following medical societies: American College of Physicians, American College of Rheumatology

Disclosure: Nothing to disclose.

Fatima A Alnaimat, MBBS Rheumatologist, Essentia Health

Fatima A Alnaimat, MBBS is a member of the following medical societies: American College of Physicians, American College of Rheumatology

Disclosure: Nothing to disclose.

Acknowledgements

Augusto C Posadas, MD Rheumatologist, Saguaro Physician Group, Tucson, AZ

Disclosure: Abbott Honoraria Speaking and teaching

Sign/Symptom	Points
Genital aphthosis	2
Oral aphthosis	2
Skin lesions	2
Neurologic manifestations	1
Vascular manifestations	1
Positive pathergy test (optional)	1

Behcet Disease Differential Diagnoses

Diagnostic Considerations

Ophthalmologic considerations

Differential Diagnoses

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