kimura 's disease is a rare benign haematological disorder , involving subcutaneous tissue , predominantly in the head and neck region and frequently associated with painless lymphadenopathies . the disease is endemic in asia , but rare and sporadic in the rest of the world . it can be associated with eosinophilia and increased total serum immunoglobulin e ( ige ) concentration . renal manifestations include membranous glomerulopathy , mesangioproliferative glomerulonephritis , minimal change disease   and focal segmental glomerulosclerosis . the aetiology is unknown , but several reports suggest a t - cell disorder or an immunoallergic reaction . we report the case of a young caucasian man presenting with the nephrotic syndrome , kimura 's disease , hyper ige and immune thrombocytopenia with signs of humoral autoimmunity . a 29-year - old caucasian man was referred for nephrotic syndrome . in the last 2 months , he had received treatment with amoxicilline clavulanate and prednisone ( 1 mg / kg / day during 5 days ) for common acquired pneumonia . cmv , parvovirus b19 , htlv1 serologies were negative , and ebv tests showed past infection , without replication . antinuclear antibodies were positive ( 1/500 ) . anti - dna , anti - ect , bone - marrow aspirate showed a slight hypereosinophilia ( 3% ) and the presence of megakaryocytic lineage , which confirmed peripheral immune thrombocytopenia ( itp ) . total plasmatic ige concentration was dramatically increased ( 3900 ui / ml , n < 20 ) . a 18-fluoro - deoxy - glucose positron emission tomography ( fdg - pet ) was negative . histologic analysis and immunohistochemistry ( cd3 , cd5 , cd10 , cd15 , cd30 , cd20 , ema , ebv - lmp , bcl2 ) excluded tumoral proliferation . we found a preserved nodal architecture with hyperplastic follicules ( figure not shown ) , many eosinophilic infiltrates and vascular hyperplasia consistent with kimura disease ( figure 1 ) . no clonal t - cell rearrangement was found , neither in the peripheral blood lymphocytes nor in the lymph node . lymph node : paracortical zone showing interfollicular infiltrates with many eosinophils ( thin arrow ) mixed with small lymphocytes . the renal biopsy specimen contained 18 glomeruli , and revealed segmental glomerular sclerosis in two of them ( figure 2 ) . there was no mesangial proliferation , nor evidence of capillary basement membrane thickening or membranous spikes . a diagnosis of kimura 's disease associated with focal segmental glomerulosclerosis ( fsgs ) and immune thrombocytopenia was retained . the patient was treated with oral corticosteroids ( prednisone , 1 mg / kg / day ) , associated with an ace inhibitor ( ramipril ) . after 1 month of treatment , while the nephrotic syndrome was in remission , antinuclear antibodies increased ( 1/1600 ) , and anti - dna antibodies appeared ( igg : 175 complement component c4 decreased to 0.10 g / l , whereas c3 remained in the normal range ( 0.85 the nephrotic syndrome relapsed twice , 12 and 18 months after the onset of steroid therapy when he was still receiving 20 mg prednisone per day . so far , the patient has no sign of malignant lymphoproliferative disorder , and no systemic autoimmune disease . kimura 's disease is a rare haematological disorder of unknown origin , occurring in young men of asian origin . pathological features include preserved lymph node architecture , proliferative germinal centres , eosinophilic infiltration and postcapillary venule proliferation , which all were present in our case . although peripheral eosinophilia was absent , most likely because of recent steroid treatment , our patient still had a slight medullar eosinophilia ( 3% ) . kimura 's disease should not be confused with angiolymphoid hyperplasia with eosinophilia ( alhe ) . this currently presents as a head or neck soft tissue swelling , with eosinophilic infiltrates and vascular proliferation . other differential diagnosis include angioimmunoblastic t - cell lymphoma , castleman 's disease , parasitic lymphadenitis and drug - related hypersensitivity lymphadenopathy . a total of 1216% of the patients present with proteinuria , and 5978% of them have the nephrotic syndrome . the skin or lymph node lesions may precede the renal manifestations , or occur simultaneously or months later , but they are usually close in time , suggesting a pathogenic link between kimura 's disease and the renal manifestations . an immunohistochemical study performed on 21 mass lesions   failed to disclose any infectious agent and clonal proliferation . no clonal rearrangement of t - cell receptor and ig heavy chain genes was found . eosinophilia and high ige levels point to a th2 cell disturbance with hypersensitivity - type reaction . .   found high il-5 levels and il-5/ifn- ratio , while katagiri et al . showed increased il-4 , il-5 and il-13 mrna expression in mononuclear cells , suggesting a role of th2 cytokines in the pathogenesis of kimura 's disease . in minimal change disease ( mcd ) also , there seems to be a th2 polarization   that suggests that in kimura 's disease - related mcd and fsgs , the th2 imbalance may play an important role . although kimura 's disease is not primarily a b - cell disorder , it is conceivable that the th2 imbalance may be associated with the production of autoantibodies in some patients . thus , efficacy of rituximab in some case of recurrent fsgs   may point to a role of b - cell disturbance in the pathogenesis of the nephrotic syndrome . treatment of kimura 's disease consists in surgical resection of the lymph node , but recurrence may occur over time . additional medical therapies , such as radiation or corticosteroids , have been used with good clinical response , but relapses are common when steroids are withdrawn . in summary , we present here the first case of kimura 's disease with fsgs , immune thrombocytopenia and signs of b - cell autoreactivity in a non - asian adult man .