Maladie de Kimura associée à une glomérulonéphrite extra-capillaire nécrosante

Abstract

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. 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. 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.