Vasculitis
Vasculitis
The vasculitides comprise a heterogeneous group of diseases with the common histopathologic feature of inflammation and necrosis of blood vessel walls. The etiologic factors associated with triggering of endothelial injury include: infections, drugs, autoantibodies, malignancies, abnormal circulating proteins, and underlying systemic disease. Half of all cases, however, do not present a clear etiology or association and are considered idiopathic. The varied causes coupled with the myriad of clinical presentations and an imperfect classification system can make the diagnosis of specific forms of vasculitis difficult. Physicians need to integrate clinical assessment with laboratory data in order to differentiate between the disorders where the vasculitic process represents a benign, self-limited disease from those that may be severe or even life threatening. Rapid and accurate diagnosis leads to optimal treatment options.
Vasculitis is defined as inflammation and fibrinoid necrosis of the blood vessel wall. It may present in a wide variety of clinical manifestations depending on the localization and size of the vessels involved, type of inflammatory infiltrate, and associated conditions. The variety of clinical presentations associated with the many etiologies of vasculitis may make the diagnosis of specific forms of vasculitis difficult. This is important, as some vasculitides with similar clinical presentation have very different prognoses and treatments. Palpable purpura, the most common clinical presentation of vasculitis, may represent a benign and self-limited disease, as is the case of some drug eruptions, or it may be a part of a life-threatening entity as in microscopic polyangiitis. Recent advances, however, have contributed significantly to our understanding of this heterogeneous group of diseases, allowing for more accurate diagnosis and optimal treatment regimens. This article will focus on syndromes and associated conditions of cutaneous necrotizing vasculitis, as well as vasculitis syndromes.
Abstract and Introduction
Abstract
The vasculitides comprise a heterogeneous group of diseases with the common histopathologic feature of inflammation and necrosis of blood vessel walls. The etiologic factors associated with triggering of endothelial injury include: infections, drugs, autoantibodies, malignancies, abnormal circulating proteins, and underlying systemic disease. Half of all cases, however, do not present a clear etiology or association and are considered idiopathic. The varied causes coupled with the myriad of clinical presentations and an imperfect classification system can make the diagnosis of specific forms of vasculitis difficult. Physicians need to integrate clinical assessment with laboratory data in order to differentiate between the disorders where the vasculitic process represents a benign, self-limited disease from those that may be severe or even life threatening. Rapid and accurate diagnosis leads to optimal treatment options.
Introduction
Vasculitis is defined as inflammation and fibrinoid necrosis of the blood vessel wall. It may present in a wide variety of clinical manifestations depending on the localization and size of the vessels involved, type of inflammatory infiltrate, and associated conditions. The variety of clinical presentations associated with the many etiologies of vasculitis may make the diagnosis of specific forms of vasculitis difficult. This is important, as some vasculitides with similar clinical presentation have very different prognoses and treatments. Palpable purpura, the most common clinical presentation of vasculitis, may represent a benign and self-limited disease, as is the case of some drug eruptions, or it may be a part of a life-threatening entity as in microscopic polyangiitis. Recent advances, however, have contributed significantly to our understanding of this heterogeneous group of diseases, allowing for more accurate diagnosis and optimal treatment regimens. This article will focus on syndromes and associated conditions of cutaneous necrotizing vasculitis, as well as vasculitis syndromes.
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