Faculty Bibliography

Systemic and localized vasculitis affects the skin and subcutis, due to large vascular beds and hemodynamic factors, such as stasis in lower extremities, and environmental influences, as occur in cold exposure. Initial cutaneous manifestations of vasculitides include diverse and dynamic patterns of discoloration, swelling, hemorrhage, and necrosis. One-half of affected patients present with localized, self-limited disease to the skin without any known trigger or associated systemic disease, known as idiopathic cutaneous leukocytoclastic vasculitis. Skin biopsy and dermatopathology contribute relevant information; however, they require correlation with clinical history, physical examination, and laboratory findings to reach an accurate diagnosis.

"Neuroimaging plays a vital role in the diagnosis of primary and secondary vasculitic disorders. There multiple neuroimaging options available to accurately describe the underlying clinical deficits of involved cases. Noninvasive neuroimaging modalities provide less risk and when interdigitated, form the basis for a more conclusive understanding of the disease process. There are instances in which invasive cerebral angiography may be needed to image the intricate and at times, small involved vessels. Neuroradiologists should be included in the multidisciplinary team of physicians caring for patients with vasculitides and in research to provide more sensitive and safe modalities for accurate diagnosis."

Illicit drug abuse is a common differential diagnosis of acquired central nervous system vasculitis even though there are only a handful of histopathologically confirmed patients in the literature from among the many potential classes of abused drugs traditionally implicated in this disease. This article considers the major classes of illicit drugs in those with and without human immunodeficiency virus type-1 infection and acquired immune deficiency syndrome.

There has been extraordinary research in the blood-brain barrier. Once considered a static anatomic barrier to the traffic of molecules in and out of the central nervous system when fully developed in adults, the blood-brain barrier is now known to be not only fully functional in development but also vital in cerebrovascular angiogenesis. Blood-brain barrier breakdown has been recognized as an important factor in a variety of primary neurologic diseases; however, such disturbances have yet to be critically analyzed. This article reviews the history, neurodevelopment, ultrastructure, function, and clinicopathologic correlation and relevance to central nervous system vasculitis.

Granulomatous inflammation, the prototypical histopathology of adult and childhood vasculitis, is characterized by inflammation of blood vessels accompanied by giant cells and epithelioid cells in the walls of cerebral vessels ranging from small leptomeningeal veins to large named cerebral arteries. Headache, hemiparesis, mental changes, abnormal cerebrospinal fluid protein content, and pleocytosis are suggestive features that warrant brain and leptomeningeal biopsy to make the diagnosis certain and begin cytotoxic therapy to improve outcome.

The epidemiology of vasculitis has witnessed extraordinary advances in the past decade influenced by the worldwide increased recognition and accurate classification and diagnosis of the vasculitides, and insights brought by genome-wide association studies and other genetic investigations that contribute to the understanding of the heritable factors of some of the disorders. This article reviews the current knowledge of the epidemiology of vasculitides in different global regions.