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Chronic granulomatous disease (CGD), also known as Bridges–Good syndrome, chronic granulomatous disorder, and Quie syndrome, [1] is a diverse group of hereditary diseases in which certain cells of the immune system have difficulty forming the reactive oxygen compounds (most importantly the superoxide radical due to defective phagocyte NADPH oxidase) used to kill certain ingested pathogens. [2]
The disease is still sometimes confused with lethal midline granuloma and lymphomatoid granulomatosis, both malignant lymphomas. [ 34 ] The full clinical picture was first presented by Friedrich Wegener (1907–1990), a German pathologist , in two reports in 1936 and 1939, leading to the eponymous name Wegener's granulomatosis or Wegener ...
The additional cells are sometimes a clue to the cause of the granuloma. For example, granulomas with numerous eosinophils may be a clue to coccidioidomycosis or allergic bronchopulmonary fungal disease, and granulomas with numerous neutrophils suggest blastomycosis, granulomatosis with polyangiitis, aspiration pneumonia, or cat-scratch disease.
Sarcoidosis (/ ˌ s ɑːr k ɔɪ ˈ d oʊ s ɪ s /; also known as Besnier–Boeck–Schaumann disease) is a disease involving abnormal collections of inflammatory cells that form lumps known as granulomata. [2] The disease usually begins in the lungs, skin, or lymph nodes. [2]
Giant cell arteritis, [6] also known as temporal arteritis or cranial arteritis, is the most common MGC-linked disease. This type of arteritis causes the arteries in the head, neck, and arm area to swell to abnormal sizes. Although the cause of this disease is not currently known, it appears to be related to polymyalgia rheumatica. [7]
Palisaded neutrophilic and granulomatous dermatitis (PNGS) is usually associated with a well-defined connective tissue disease, lupus erythematosus or rheumatoid arthritis most commonly, and often presents with eroded or ulcerated symmetrically distributed umbilicated papules or nodules on the elbows.
Eosinophilic granulomatosis with polyangiitis consists of three stages, but not all patients develop all three stages or progress from one stage to the next in the same order; [7] whereas some patients may develop severe or life-threatening complications such as gastrointestinal involvement and heart disease, some patients are only mildly affected, e.g. with skin lesions and nasal polyps. [8]
Lymphomatoid granulomatosis (LYG or LG) is a very rare lymphoproliferative disorder first characterized in 1972. [1] Lymphomatoid means lymphoma-like and granulomatosis denotes the microscopic characteristic of the presence of granulomas with polymorphic lymphoid infiltrates and focal necrosis within it.