Chenthilnathan PERİASAMY, Norzaliana ZAWAWİ, Salleh Md Salzihan MD, Baharudin ABDULLAH

Kimuras disease an unusual cause of lymphadenopathy in a nephrotic syndrome child

Kimura hastalığı, doğuda endemik olan etyolojisi bilinmeyen nadir, bening, kronik inflamutuar bir hastalıktır. Hastalık, baş ve boyun bölgesinde ağrısız subkütanöz nodül, eozinofili ve yüksek serum immunoglobulin E (IgE) düzeyleri üçlüsü ile karakterizedir. Kimura hastalığının, eozinofilik lenfogranuloma, inflammatuar anjiomatöz nodül ve atipik pyojenik granuloma gibi çeşitli sinonimleri vardır. Kimura hastalığı ve renal lezyon arasında ortak etyopatogenezi oluşturan nefrotik sendrom gibi renal patoloji Kimura hastalığı ile birlikte bulunur. Biz, sol kulak arkası şişliği olan tekrarlamalı nefrotik sendromlu 11 yaşında Malezyalı bir erkek çocuğu sunuyoruz. Diyagnoz, cerrahi biyopsi sonrasındaki karakteristik histopatoloji bulgularına dayanarak Kimura hastalığı idi.

Anahtar Kelimeler:

İmmunoglobulin E, Kimura hastalığı, lenfadenopati, nefrotik sendrom, periferal eozinofili

Nefrotik sendromlu bir çocukta lenfadenopatinin olağandışı nedeni olarak Kimura hastalığı

Kimuras disease is a rare, benign, chronic inflammatory disorder of unknown etiology which is endemic in Orientals. The disease is characterized by a triad of painless subcutaneous nodule in the head and neck region, blood eosinophilia and elevated serum immunoglobulin E (IgE) levels. Kimuras disease has various synonyms such as eosinophilic lymphogranuloma, inflammatory angiomatous nodule and atypical pyogenic granuloma. Renal pathology such as nephrotic syndrome has been found to be associated with Kimuras disease which states a common etiopathogenesis between renal lesion and Kimuras disease. We present an eleven years old Malay boy with recurrent nephrotic syndrome presented with left post auricular swelling. The diagnosis was based on the characteristic histopathology findings after surgical biopsy as Kimuras disease.

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