E. Miscellaneous Vasculitis (Behçet’s Disease, Primary Angiitis of the Central Nervous System, Cogan’s Syndrome, and Erythema Elevatum Diutinum)
  • Kenneth T. Calamia
  • Carlo Salvarani


  • The prevalence of Behçet’s disease is highest in countries of the eastern Mediterranean, the Middle East, and East Asia.

  • Aphthous oral ulcers are usually the first and most persistent clinical feature of Behçet’s disease. Aphthous ulcers also occur frequently on the genitals (e.g., the scrotum or vulva).

  • Uveitis-either anterior or posterior-is common in Behçet’s disease and a source of major morbidity.

  • Many forms of central nervous system disease may occur in Behçet’s disease. These include aseptic meningitis and white matter lesions in the brainstem.

  • Human leukocyte antigen (HLA)-B51 is a strong risk factor for Behçet’s disease.

  • The diagnosis of primary angiitis of the central nervous system is predicated upon either biopsy evidence of vasculitis or angiographic findings suggestive of vasculitis in the setting of other compelling features, for example, strokes demonstrated by magnetic resonance imaging or the findings of a cerebrospinal fluid pleocytosis.

  • The diagnosis of primary angiitis of the central nervous system should never be made on the basis of an angiogram alone.

  • Patients with benign angiopathy of the central nervous system are predominantly female, tend to present acutely with headache (with or without focal symptoms), and have normal or near normal cerebrospinal fluid.

  • Cogan’s syndrome refers to the association of inflammation in both the eyes and ears: specifically, the occurrence of nonsyphilitic interstitial keratitis and immune-mediated inner ear disease, resulting in audiovestibular dysfunction.

  • Any type of ocular inflammation may occur in Cogan’s syndrome (e.g., scleritis, uveitis, orbital pseudotumor). The inner ear disease associated with this condition often leads to deafness.

  • ■ In erythema elevatum diutinum, skin lesions consist of purple, red, or brown plaques and often have an annular or nodular appearance. The skin lesions have a predilection for the extensor surfaces of the distal extremities and often overlie joints, but may be generalized.


Central Nervous System Disease Cerebral Venous Thrombosis Retinal Vasculitis Pulmonary Artery Aneurysm Primary Angiitis 
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Copyright information

© Springer Science+Business Media, LLC. 2008

Authors and Affiliations

  • Kenneth T. Calamia
    • 1
  • Carlo Salvarani
    • 2
  1. 1.Department of Medicine, Division of RheumatologyMayo Clinic College of MedicineJacksonvilleUSA
  2. 2.Division of RheumatologyHospital S. Maria NuovaReggio EmiliaItaly

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