Behçet’s Disease. Differential Diagnosis

  • Maria Grazia SabbadiniEmail author
  • Stefano Franchini
Part of the Rare Diseases of the Immune System book series (RDIS)


Behçet’s disease (BD) is a systemic inflammatory disorder characterized by a very wide spectrum of clinical features. Although oral ulceration, genital ulceration, and eye disease represent the classic triad of manifestations, the cardiovascular, gastrointestinal, musculoskeletal, and central nervous systems can also be affected. Thus the differential diagnosis of BD is complicated by the panopoly of clinical symptoms and signs and also by the unpredictable cycles of remission and exacerbation. Furthermore diagnosing BD, often incurs a considerable delay due to the rarity of this condition, particularly outside of endemic regions. The diagnosis of this condition is only supported by clinical criteria that require the exclusion of other diagnoses based on clinical presentation. Indeed, since neither the laboratory data nor the histopathological signs are truly pathognomonic in BD, the differential diagnosis depends on a careful evaluation of the medical history and meticulous physical examination to detect concomitant systemic manifestations. Owing to the vast array of clinical features possibly presented by the patients affected by this disease, the differential diagnosis of BD includes recurrent aphthous stomatitis, viral infections, Reiter’s syndrome, systemic lupus erythematosus, Stevens-Johnson’s syndrome, inflammatory bowel disease, PFAPA syndrome, MAGIC syndrome, Sweet’s syndrome, sarcoidosis, multiple sclerosis, ankylosing spondylitis, and many other conditions.


Mucocutaneous manifestations Articular manifestations Gastrointestinal manifestations Ocular manifestations Nervous system involvement Cardiovascular disease 


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Copyright information

© Springer-Verlag Italia 2014

Authors and Affiliations

  1. 1.General Medicine and Clinical ImmunologyOspedale San Raffaele, and Università Vita-Salute San RaffaeleMilanItaly
  2. 2.General Medicine and Clinical ImmunologyOspedale San RaffaeleMilanItaly

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