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                      Kimura's disease 4

                             

 

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Kimura's Disease

Visit:  Dermpath-India

Kimura's disease associated with extracapillary glomerulonephritis. Ann Dermatol Venereol. 2006 Aug-Sep;133(8-9 Pt 1):689-92.

BACKGROUND: Kimura's disease is a rare and benign chronic inflammatory soft-tissue disorder of unknown origin. Most cases involve young Asian males. The typical presentation consists of painless papules or nodules with a predilection for the head and neck region, associated with lymphadenopathy, parotid gland involvement, peripheral blood eosinophilia and raised immunoglobulin E. PATIENTS AND METHODS: A 49-year-old Mauritian man was hospitalized for nephrotic syndrome. Examination revealed a painless 3 cm nodule in the left pre-auricular region and multiple enlarged left cervical lymph nodes. Histopathologic examination of a nodule confirmed the diagnosis of Kimura's disease, which was associated with necrotic extracapillary glomerulonephritis. Corticosteroids were initiated, resulting in rapid improvement of renal function and partial regression of the patient's cutaneous nodules and lymph node disorder. DISCUSSION: Renal manifestations are the only visceral localization of Kimura's disease, and proteinuria is seen in 12% of cases, of which 50% are accompanied by nephrotic syndrome. Many different histologic types of renal involvement may be observed with potentially severe lesions. To our knowledge, this is the first case of Kimura's disease associated with extracapillary glomerulonephritis.

Clinicopathologic study of parotid involvement in 21 cases of eosinophilic hyperplastic lymphogranuloma (Kimura's disease). Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2006 Nov;102(5):651-8. Epub 2006 Apr 21.

OBJECTIVE: To investigate the clinicopathologic features of eosinophilic hyperplastic lymphogranuloma (Kimura's disease) in the parotid gland. STUDY DESIGN: The hematoxylin and eosin sections and clinical data of 60 patients with eosinophilic hyperplastic lymphogranuloma (Kimura's disease), including with parotid involvement, were reviewed. RESULTS: Of 60 cases of eosinophilic hyperplastic lymphogranuloma (average age, 42 years; average disease duration, 5.8 years), 35 cases (58%) were clinically seen to involve swelling of the parotid region. Parotid specimens were available in 21 cases and showed different microscopic changes. In mildly affected parotid samples, the histological features included infiltration of lymphocytes and eosinophils around the ducts of the interlobular connective tissues. In the moderately involved glands, the infiltrated area was enlarged and contained lymphoid follicles, resulting in adjacent acinar atrophy that was particularly obvious around the salivary ducts. In severe lesions, most acini were lost and only a few ducts remained. All cases with parotid involvement showed more severe pathological changes in the subcutaneous connective tissues and/or local lymph nodes. The parotid lesions often surrounded a central intraglandular lymph node with characteristic features of the disease; however, the salivary parenchyma was left alone. Nerve fibers affected by inflammatory lymphocytes and eosinophils were seen in 38/60 (63%) cases of eosinophilic hyperplastic lymphogranuloma (Kimura's disease) examined in this study. CONCLUSIONS: Eosinophilic hyperplastic lymphogranuloma (Kimura's disease) does not show primary parotid involvement, but instead, pathological changes in the parotid gland are a result of disease spread from the intraparotid lymph nodes and adjacent soft tissues. In addition, our observations suggest that the pruritus often associated with the disease may be due to nerve infiltration by lymphocytes and eosinophils.

 
July 2009 
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