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The definite diagnosis of vasculitis is established after a biopsy of involved organ or tissue, such as skin, sinuses, lung, nerve, brain, and kidney. The biopsy elucidates the pattern of blood vessel inflammation. Some types of vasculitis display leukocytoclasis, which is vascular damage caused by nuclear debris from infiltrating neutrophils. [37]
The disease spectrum varies from failure of multiple organs to involvement of a single organ. Almost any organ could be impacted; however, polyarteritis nodosa rarely affects the lungs for unknown reasons. [10] Kawasaki disease (KD) is a type of systemic vasculitis of medium-sized vessels with an acute onset that primarily affects young children.
The term childhood disease refers to disease that is contracted or becomes symptomatic before the age of 18 or 21 years old. Many of these diseases can also be contracted by adults. Some childhood diseases include:
In cases where a cause can be determined, medications and infectious pathogens are most common in adults, while IgA vasculitis (Henoch–Schönlein purpura) frequently affects children. [6] Other etiologies include autoimmune conditions and malignancies, usually hematologic (related to the blood). [5] [6]
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Kawasaki disease (also known as mucocutaneous lymph node syndrome) is a syndrome of unknown cause that results in a fever and mainly affects children under 5 years of age. [6] It is a form of vasculitis, in which medium-sized blood vessels become inflamed throughout the body. [1]
IgA vasculitis (HSP), previously known as Henoch–Schönlein purpura, is an autoimmune disease that most commonly affects children. In the skin, the disease causes palpable purpura (small, raised areas of bleeding underneath the skin), often with joint pain and abdominal pain .
Several rare conditions are due to defects in the innate immune system, which is a basic line of defense that is independent of the more advanced lymphocyte-related systems. Many of these conditions are associated with skin problems. [7] Interleukin 12 receptor, beta 1 deficiency; IL-12p40 deficiency; Interferon gamma receptor 1 deficiency