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The signs and symptoms of GPA are highly varied and reflect which organs are supplied by the affected blood vessels. Typical signs and symptoms include nosebleeds, stuffy nose and crustiness of nasal secretions, and inflammation of the uveal layer of the eye. [3] Damage to the heart, lungs and kidneys can be fatal. The cause of GPA is unknown.
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]
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]
Specifically, thoracic spine and frontal bone within the skull are the most affected in children. On the other hand, cervical spine and jaw bone are the major spots of EG lesions in adults. Symptoms of EG include stiffness, local pain, edema of surrounding tissues, posture change, and many others, depending on the affected bone. [1]
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.
LCH usually affects children between 1 and 15 years old, with a peak incidence between 5 and 10 years of age. Among children under the age of 10, yearly incidence is thought to be 1 in 200,000; [50] and in adults even rarer, in about 1 in 560,000. [51] It has been reported in elderly but is vanishingly rare. [52]
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.
Palisaded neutrophilic and granulomatous dermaititis is associated with subacute bacterial endocarditis, [4] ledipasvir/sofosbuvir, [5] allopurinol, [6] Hodgkin’s and non-Hodgkin’s lymphoma, [4] chronic myelomonocytic leukemia, [7] ulcerative colitis, [8] Takayasu arteritis, [4] systemic vasculitis, [8] systemic lupus erythematosus (SLE), [9] sarcoidosis, [10] rheumatoid arthritis, [11 ...