Cases reported "Fibrosis"

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1/4. Sinonasal tract eosinophilic angiocentric fibrosis. A report of three cases.

    Eosinophilic angiocentric fibrosis (EAF) is a rare submucosal fibrosis without a well-developed differential diagnosis. Three cases of sinonasal tract EAF were identified in 2 women and 1 man, aged 49, 64, and 28 years, respectively. The patients experienced a nasal cavity mass, maxillary pain, or nasal obstructive symptoms of long duration. The process involved the nasal septum (n = 2), nasal cavity (n = 1), and/or the maxillary sinus (n = 1). There was no evidence for wegener granulomatosis, churg-strauss syndrome, Kimura disease, granuloma faciale, or erythema elevatum diutinum. Histologically, the lesions demonstrated a characteristic perivascular "onion-skin" fibrosis and a full spectrum of inflammatory cells, although eosinophils predominated. necrosis and foreign body-type giant cells were not identified. Surgical excision was used for all patients, who are all alive but with disease at last follow-up. Sinonasal tract EAF is a unique fibroproliferative disorder that does not seem to have systemic associations with known diseases. The characteristic histomorphologic features permit accurate diagnosis.
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ranking = 1
keywords = granulomatosis
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2/4. Eosinophilic angiocentric fibrosis and Wegener's granulomatosis: a case report and literature review.

    This report presents a case of eosinophilic angiocentric fibrosis in a man with Wegener's granulomatosis, the first report of a possible association between the two conditions. This association suggests a possible mechanism for its pathogenesis.
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ranking = 5
keywords = granulomatosis
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3/4. Eosinophilic angiocentric fibrosis.

    Eosinophilic angiocentric fibrosis (EAF) is a rare disease of the sinonasal tract, with histologic characteristic features like thick collagen bundles whirling around vessels in a fibrotic stroma with inflammatory cells rich in eosinophils. The Authors present a case of a 31-year-old man with bilateral nasal obstruction with no history of allergies or other systemic disease. The patient underwent a septoplasty with symptoms relieving. An EAF diagnosis was made. Differential diagnosis must rule out other lesions that may mimic EAF such as granuloma faciale, Kimura disease, wegener granulomatosis, churg-strauss syndrome.
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ranking = 1
keywords = granulomatosis
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4/4. Wegener's granulomatosis, acute laryngotracheal airway obstruction and death in a 17-year-old female: case report and review of the literature.

    OBJECTIVE: To alert practitioners to the risk of sudden airway obstruction and death in patients with Wegener's granulomatosis. DESIGN: Case report and literature review. SETTINGS: University and Community hospitals. PATIENT: A 17-year-old white female. INTERVENTION: (1) evaluation and treatment for mental status changes over 2 months. (2) Evaluation and surgical biopsy of nasal septal perforation under general anesthesia at a university children's hospital. (3) Evaluation and observation at a community hospital 2 days later. (4) autopsy. RESULTS: serology performed 3 days ante-mortem revealed (at 2 days post-mortem) cytoplasmic anti-neutrophil cytoplasmic antibody positive at 1:128. autopsy was significant for microscopic fibrosis and granulomas in the kidneys and essentially total obstruction of the subglottis and upper trachea by a 3.5 x 1 x 1 cm mass of fibrosis and granulomas overlying circumferentially necrotic mucosa. This mass was centered on the crico-tracheal junction. CONCLUSIONS: Wegener's granulomatosis can lead to proliferative tissue growth with acute airway obstruction in the larynx and trachea, and death. Any patient with WG under age 20, as well as patients with WG and laryngotracheal symptoms (e.g. strider, hoarseness, wheezing) would benefit from evaluation of the airway.
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ranking = 6
keywords = granulomatosis
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