Cases reported "Mikulicz' Disease"

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1/3. Mikulicz's disease and Mikulicz's syndrome: analysis of the original case report of 1892 in the light of current knowledge identifies a MALT lymphoma.

    OBJECTIVE: To remove the confusion surrounding the terms "Mikulicz's disease" and "Mikulicz's syndrome" by discovering the nature of the disease that Mikulicz described. STUDY DESIGN: Investigation of the original 1892 publication by Mikulicz in the light of current knowledge. RESULTS: Mikulicz described bilateral painless swellings of the lacrymal, major salivary, and minor salivary glands that were present for 14 months without a reduction in salivary flow before the patient, a man of 42 years, died. A print of a microscopical field of one of the submandibular glands contains a uniform lymphocytic infiltrate of centrocyte-like cells typical of MALT lymphoma, which is strongly supported by the reported clinical features. CONCLUSION: Mikulicz published the first case report of a MALT lymphoma of salivary glands, and the terms "Mikulicz's disease" and "Mikulicz's syndrome" should no longer be used.
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ranking = 1
keywords = submandibular gland, submandibular
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2/3. Mikulicz's disease: a new perspective and literature review.

    PURPOSE: To report the clinical and pathophysiologic features of two patients with Mikulicz's disease and to further characterize recommendations for diagnosis and management with a review of the literature. methods: Retrospective nonrandomized consecutive case series, Jules Stein Eye Institute, David Geffen School of medicine at UCLA. RESULTS: Mikulicz's disease is characterized by symmetric lacrimal, parotid, and submandibular gland enlargement with associated lymphocytic infiltrations. The authors noted two cases of Mikulicz's disease. The diagnosis of Mikulicz's disease was based on the following criteria: 1) symmetric and persistent swelling of the lacrimal glands and either or both of the major salivary glands (parotid and submandibular); and 2) the exclusion of other diseases that may mimic this presentation, such as sarcoidosis, viral infection, or lymphoproliferative disorders. CONCLUSIONS: Mikulicz's disease is a condition in which there is bilateral lacrimal and salivary gland swelling that is not associated with other systemic conditions. The condition is self-limiting and most often, the diagnosis is a clinical one. Previously, Mikulicz's disease was often considered as a subtype of sjogren's syndrome (SS). Clinical and immunologic differences between Mikulicz's disease and SS may warrant further consideration of Mikulicz's disease as a specific autoimmune phenomenon separate from SS, and Mikulicz's disease may be amenable to different treatment modalities than those employed in patients with SS.
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ranking = 1.1446039550671
keywords = submandibular gland, submandibular
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3/3. A case of Mikulicz's disease complicated with interstitial nephritis successfully treated by high-dose corticosteroid.

    A 40-year-old woman who had bilateral swelling in the eyelids and submandibular region was admitted. Clinical findings suggested she had primary sjogren's syndrome. Laboratory data showed glucosuria, positive CRP (0.50 mg/dl), liver dysfunction (AST 53 U/l, ALT 101 U/l, gamma-GTP 241 U/l, ALP 914 U/l, LAP 496 U/l), hyperglycemia, hypergammaglobulinemia (IgG 3450 mg/dl, IgA 91 mg/dl, IgM 80 mg/dl), hypocomplementemia (C3 73 mg/dl, C4 2 mg/dl, CH50 < 19.0 U/ml), renal tubular dysfunction (urine N-acetyl-beta-D: -glucosaminidase 8.6 U/l, urine (beta2)-microglobulin 83 microg/l), and urinary concentration defect. ammonium chloride loading test was normal. gallium-67 scintigram indicated abnormal uptake in bilateral lacrimal glands, submandibular glands, and kidneys. A diagnosis of Mikulicz's disease and interstitial nephritis was made, since biopsy specimens of her lacrimal gland and minor salivary gland showed diffuse infiltration of lymphocytes. Renal biopsy specimens also showed severe interstitial infiltration of lymphocytes. Symptoms and laboratory data normalized in response to methylprednisolone pulse therapy and prednisolone 60 mg/day. This case of Mikulicz's disease complicated with interstitial nephritis was successfully treated by high-dose corticosteroid.
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ranking = 1.1446039550671
keywords = submandibular gland, submandibular
(Clic here for more details about this article)


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