死の転帰をとつた鼻悪性肉芽腫症4例 : Wegener肉芽腫症の定義に当てはまらない進行性鼻壊疽

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Four cases of lethal granuloma of the nose, orbit, and palate were reported. These cases revealed the local signs and findings resembled those of Wegeners granulomatosis but were considered as its variant because incompletion of the symptoms. Case 1: A window, aged 53, who had right exophthalmus for one year and had become well by steroid therapy, was referred to our clinic with nasal bleeding and rhinorrhaa. Biopsy specimens from nose and paranasal sinus revealed non-specific inflammation. But a few weeks later, she developed hematuria and anuria. Subacute glomeruritis was diagnosed. She died one year and ten months after the onset of the disease. Case 2: A house wife, aged 59, who had had rheumatic arthritis for two years and had been treated with cortico-steroid, developed left purulent rhinorrhea with blood. Rhinoscopy revealed nasal septal perforation and granulation. Repeated biopsies revealed inflammatory and granulomatous changes with necrosis and the proliferation of atypical reticulum cells.The lesion increased its size and the palate was perforated. One year and five months later, she died due to prostration and bleeding from the lesion. Case 3: A man, aged 55, who had had pulmonary tuberculosis and bronchial asthma, developed nasal obstruction, swelling of the cheek, and fever. Biopsy specimen from the lesion did not reveal evident vasculitis. The hard palate underwent necrotic changes despite steroid and radiation therapy. The patient died four months after the onset of the disease. Case 4: A house wife, aged 50, developed hemorrhagic rhinorrhea, swelling of the root of the nose, and fever. Histogical findings showed inflammation with necrosis. Vasuculitis was not observed. After radiation therapy, the necrotic changes of the palate developed. In the terminal stage, the abdominal distention was recognized. Autopsy findings revealed the infiltration of mononucler histiocytes to liver, kidney, lung and other organs. It should be recognized that there are some lethal granulomatous and necrotic disease arising from the nose, orbit and palate without any specific infection. These cases are not diagnosed as Wegeners granulomatosis but it is considered that there are common pathogenic factors for these non-healing granuloma.

死の転帰をとつた鼻悪性肉芽腫症4例 : Wegener肉芽腫症の定義に当てはまらない進行性鼻壊疽

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