Nephrol Dial Transplant (1996) 11: 1349-1351 Case Report Nephrology Dialysis Transplantation Kimura's disease and minimal-change nephrotic syndrome K. Sud 1 , T. Saha 1 , A. Das 2 , N. Kakkar 2 , V. Jha 1 , H. S. Kohli 1 and V. Sakhuja 1 Departments of 'Nephrology and 2 Pathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India Key words: Kimura's disease; subcutaneous eosino- philic lymphoid granuloma; nephrotic syndrome; minimal-change disease; mesangial proliferative glom- erulonephritis Introduction First described in 1937 by Kimm and Szeto in Chinese literature as 'eosinophilic hyperplastic lymphogranul- oma', this condition became widely known as Kimura's disease after Kimura et al. reported similar cases in Japan in 1948 (cited from reference [1]). This is an angiolymphoid proliferative disorder of unknown aetiology, presenting with subcutaneous tumorous nodules, with a predilection for periauricular and sub- mandibular regions. The disease occurs predominantly in oriental males and is characterized by the presence of eosinophilic lymphoid granulomas sometimes appearing as eosinophilic abscesses with variable degrees of fibrosis [1-3]. Kimura's disease has been described under various synonyms such as subcutane- ous eosinophilic lymphoid granuloma, eosinophilic fol- liculosis of the skin, inflammatory angiomatous nodule, pseudo- or atypical pyogenic granuloma and angio- lymphoid hyperplasia with eosinophilia (ALHE); however, recently ALHE has been proposed to be a separate clinicopathological entity [1]. Renal abnormalities, notably proteinuria and nephrotic syndrome have been found to be associated with Kimura's disease [4,5]. A few reports describing a steroid-responsive nephrotic syndrome have shown reduction in the size of subcutaneous nodules with steroids [6,7], implying a common aetiopathogenesis of the renal lesion and Kimura's disease. We report a patient with steroid-responsive minimal-change nephrotic syndrome who had a steroid unresponsive Kimura's disease requiring complete resection of the tumour. This is also the first reported case of Kimura's disease associated with nephrotic syndrome in a patient from the Indian subcontinent. Case report A 24-year-old Indian housewife was referred for evalu- ation of persistent proteinuria first noticed in the third trimester of pregnancy. She did not have pre-eclampsia and 6 weeks prior to her referral, she had delivered a live fetus at 34 weeks of gestation. On examination, she had anasarca and was normotensive. Investigations revealed a haemoglobin of 12.1 g/dl, with normal total and differential leukocyte counts. There was no eosino- philia. Urinalysis revealed 4 + albuminuria and 2-3 RBCs/HPF. Her 24 h urinary protein excretion was 4.1 g/day and serum creatinine was 160umol/l. She had a total serum protein of 35 g/1, a serum albumin of 15 g/1, and serum cholesterol of 16.55 mmol/1. LE cell preparation and ANF were negative and C3 levels were 1 g/1 (normal 0.7-1.6 g/1). Renal biopsy was done which showed 15 glomeruli all of normal morphology on light-microscopy. There was no mesangial prolifera- tion or expansion (Figure 1). There was no evidence of capillary basement membrane thickening on PAS stain and silver impregnation did not reveal any epi- membranous spikes. Tubules showed hyaline casts and the interstitium was normal. Immunofluorescence was negative. The patient was treated with prednisolone 1 mg/kg/day for 8 weeks to achieve remission at which time her serum creatinine was 90 umol/1 and the 24 h Correspondence and offprint requests to: Professor V. Sakhuja, Head, Department of Nephrology, Postgraduate Institute of Medical Education and Research, Chandigarh 160012, India. Fig. 1. Glomeruli showing normal morphology on light-microscopy (H&Ex240). © 1996 European Dialysis and Transplant Association-European Renal Association by guest on August 4, 2015 http://ndt.oxfordjournals.org/ Downloaded from