kimura 's disease ( kd ) is an uncommon , benign , chronic inflammatory disorder of unknown aetiology . kd typically presents as a painless subcutaneous swelling in the preauricular and submandibular regions , though involvement of the orbit , eyelids , epiglottis , axilla , forearm , groin or popliteal region has been reported . a 52-year - old female of chinese decent was referred regarding a persistent right submandibular swelling thought to be related to an odontogenic infection . history revealed 2 months of progressive painless right submandibular swelling despite several courses of oral antibiotics and recent dental extraction . she denied any infective or respiratory symptoms , weight loss or dysphagia . aside from right submandibular chronic sclerosing sialadenitis treated via surgical excision , the patient had no medical history , no known allergies and took no regular medications . clinical examination revealed a firm , non - tender , swelling of the right submandibular region with no overlying skin changes . laboratory investigations were unremarkable other than an increased eosinophil count of 1.8  10/l ( normal range 0.00.5  10/l ) and serum creatinine of 91 mol / l ( normal range 4990 mol / l ) . computed tomography ( ct ) identified several enlarged submandibular lymph nodes ( figs  1 and 2 ) . figure  1:axial ct neck ( post contrast ) showing enlarged right submandibular nodes . fine needle aspiration ( fna ) biopsy of a right submandibular lesion was non - diagnostic ; however , subsequent excisional biopsy indicated kd . the patient was referred to rheumatology and commenced on cetrizine and a tapering course of oral prednisolone . by 3 months , the submandibular swelling had clinically resolved , and at 9 months , the patient remained recurrence free with normal renal function and eosinophil count . shortly after cessation of prednisolone , submandibular swelling and eosinophilia recurred ; therefore , oral steroids were recommenced . kd most commonly , though not exclusively , occurs in young and middle - aged adults of asian descent affecting men more commonly than women . although the aetiology of kd remains uncertain , the general consensus is that kd is a chronic inflammatory condition . alternative proposed aetiologies include neoplasia , parasitic infection , atopic reaction or immune response to various pathogens , in particular , candida albicans ; however , no causative agent has been isolated in lesions of kd . differential diagnoses of kd include tuberculosis , nodal metastases , lymphoma , eosinophilic granuloma , granulomatous diseases , salivary gland pathologies , lymphadenitis , drug reaction and angiolymphoid hyperplasia with eosinophilia ( alhe ) [ 1 , 4 , 6 ] . the most controversial of these is alhe , as at times alhe and kd have been considered to be the same disease . the degree of eosinophilia may correlate with the size of lesions , and recurrences may be more likely in patients with eosinophil counts > 50% and serum ige > 10 000 iu / ml . magnetic resonance imaging and ct assist in assessing lesions and their relations to surrounding structures , with lesions often being ill defined and of variable appearance on cross - sectional imaging . lesions have been found to be diffusely avid on positron emission tomography scanning and may resemble neoplastic disorders . given the non - specific radiological findings , imaging alone is often insufficient to exclude malignancy ; thus , diagnosis is based on biopsy in conjunction with clinical features and laboratory findings . once the diagnosis of kd has been established , further imaging to assess for distant lesions is not considered essential . histologically , kd demonstrates follicular hyperplasia with reactive germinal centres , and there is often ige deposition and eosinophilic infiltration of germinal centres and interfollicular zones . lysis of follicles with microabscess formation may also be present   ( figs  3 and 4 ) . fna is generally inadequate to diagnose kd ; consequently , open biopsy is often required to provide diagnostic tissue samples . figure  3:photomicrograph of haematoxylin and eosin ( h&e)-stained sections ( 400 ) showing collection of eosinophils within the paracortex . photomicrograph of haematoxylin and eosin ( h&e)-stained sections ( 400 ) showing collection of eosinophils within the paracortex . observation may be acceptable if lesions are asymptomatic and not cosmetically concerning ; however , untreated lesions tend to slowly enlarge and may become disfiguring . reported medical treatments include corticosteroids , cetrizine , trans - retinoic acid , pentoxifyline , cyclosporine , oxyphenbutazone , vincristine and pranlukast [ 1 , 3 ] . excisional biopsy has been advocated as the initial treatment of choice as it provides tissue for histological diagnosis and may be curative . recurrence has been reported in up to 25% of patients treated with surgical excision alone . in cases of initial recurrence , reported that recurrences occurred between 1.3 and 2.5 years in patients treated with surgery , whilst zhang et al . given these reports , it would appear prudent to keep patients under review for at least 3 years following the completion of treatment to monitor for recurrence . given that kd is a non - malignant disease , radiotherapy is generally limited to cases not amenable to surgery or those who have failed medical therapies . nephrotic syndrome and renal impairment are important sequelae of kd , which may affect up to 60% of patients . patients with renal complications should be referred to a renal physician . in patients with severe renal disease , aggressive treatment with corticosteroids , cyclophosphamide and antiplatelet drugs has been recommended . in summary , all patients presenting with a progressive head and neck swelling should be thoroughly investigated to exclude malignancy . although kd is an uncommon disease , medical and dental practitioners should be aware of the condition because of its ability to become disfiguring , recur and result in renal complications . the presence of eosinophilia and elevated serum ige should suggest the possibility of kd and if suspected patients should be investigated for renal involvement and referred accordingly .
kimura 's disease ( kd ) typically presents as a mass in the head and neck region in association with eosinophilia and elevated serum ige . 
 excisional biopsy is often required in order to obtain an adequate sample for histological diagnosis and exclude malignancy . 
 if suspected , patients should also be investigated for renal involvement as this may complicate kd . 
 treatment options include surgical excision and medical therapies such as corticosteroids depending on the extent and severity of disease .