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Case No. 283
2012-09-01~2012-09-30
출제자 : 최영준
Hit : 594
| Period | 2012-09-01~2012-09-30 |
|---|---|
| Diagnosis | IgG4-related sclerosing disease |
| Clinical information | 64/M 3개월전부터 양측 턱밑에 mass 만져짐 ESR 41 mm/hr |
| Discussion | IgG4-related sclerosing diseases in the head and neck Usually middle-aged to elderly adults Site involvement - Chronic sclerosing sialadenitis, submandibular glands (Küttner’s tumor) - Lacrimal and parotid glands (Mikulicz’s disease) - Pituitary gland (lymphoplasmacytic hypophysitis) - The nasal cavity : extremely rare Serologic findings - Elevated serum IgG4 concentrations - Approximately 30% of patients have normal serum IgG4 concentrations . Image findings - Nonspecific Diffuse enlargement of bilateral salivary or lacrimal glands. Thickening of nasal septum Histopathological findings - Infiltration of many lymphocytes and IgG4-positive plasma cells (> 10/HPF) - Various degrees of fibrosclerosis - Obstructive phlebitis Usually respond well to steroid therapy |
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