Behcet disease

Aksel Siva MD (

Dr. Siva of the Istanbul University Cerrahpasa School of Medicine received honorariums from Bayer-Schering AG, Biogen/Gen Ilac of Turkey, Genzyme, Merck-Serono, and Roche for consulting, fees from Novartis as a consultant and advisory committee member, travel and registration reimbursements from Genzyme, and honorariums from Teva for speaking engagements.

)
Francesc Graus MD PhD, editor. (Dr. Graus of the University of Barcelona has no relevant financial relationships to disclose.)
Originally released October 14, 1994; last updated January 1, 2020; expires January 1, 2023

This article includes discussion of Behcet disease, Behçet's syndrome, silk route disease, triple symptom complex, multiple symptom complex of Behcet, Morbus Behcet, oculobuccogenital syndrome, Adamantiades-Behcet disease, neurobehçet, neurobehcet, neuro-Behçet, neuro-Behcet, vasculo-Behcet, entero-Behcet, incomplete Behcet disease, complete Behcet disease, mucocutaneous form of Behcet disease, and neuro-ocular form of Behcet disease. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

Behcet disease is an idiopathic chronic relapsing multisystem vascular-inflammatory disease of unknown origin that can affect the nervous system. Clinical and imaging evidence suggests that primary neurologic involvement may be subclassified into 2 major forms. The first form, which is seen in the majority of patients, may be characterized by a vascular-inflammatory central nervous system disease with focal or multifocal parenchymal involvement. The other form is caused by isolated cerebral venous sinus thrombosis and intracranial hypertension. In this article, the author describes the various clinical manifestations, summarizes the diagnosis and differential diagnosis, and gives an update on the emerging views on its epidemiology, pathogenesis, suggested diagnostic MRI patterns, and other biomarkers as well as current management issues for the neurologic disease that include the use of anti-TNF-alpha agents.

Key points

 

• Behcet disease is a disorder of unclear pathogenesis. Several inflammatory markers and the association of Behcet disease with particular major histocompatibility complex antigens point to inflammatory mechanisms inducing the manifestations of the disease in a genetically predisposed host.

 

• Neurologic involvement, or Neuro-Behcet syndrome (NBS), is heterogeneous with 2 major clinical subtypes: a central nervous system inflammatory parenchymal disease and a less common nonparenchymal form that involves large extraparenchymal vascular structures, mainly venous dural sinuses. Dural venous sinus thrombosis is not uncommon in most cases of pediatric NBS. Peripheral nervous system involvement is extremely rare.

 

• NBS is a great mimicker of various central nervous system disorders. A history of oral and/or genital ulcers and uveitis should be sought in young patients who present with stroke-like syndromes or with symptoms involving the brainstem and corticospinal tracts, as well as increased intracranial pressure, or dural sinus venous thrombosis.

 

• New anti-inflammatory agents, in particular TNF-alpha inhibitors, currently are accepted to be the most efficient therapeutic alternative to conventional immunosuppressive drugs.

Historical note and terminology

The earliest known observations of oral and genital ulcerations associated with uveitis are in Hippocrates' notes (Feigenbaum 1956). The disease was named after Dr. Hulusi Behcet (Behcet 1937).

Turkish dermatologist Dr. Hulusi Behcet Image: Dr. Hulusi Behcet
Since then, the manifestations of the disease have become more widely known and have been extended to include multiple systems.

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