Behcet Disease Differential Diagnoses

Updated: Oct 19, 2022
  • Author: Nicole Davey-Ranasinghe, MD; Chief Editor: Herbert S Diamond, MD  more...
  • Print

Diagnostic Considerations

Other conditions to consider are as follows:

  • Malignancy [46]
  • HLA-B27–associated syndromes ( ankylosing spondylitis, reactive arthritis, psoriatic arthritis)
  • Inflammatory bowel disease
  • 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