kikuchi - fujimoto disease ( kfd ) is a benign , self - limited , inflammatory disorder , first reported in japan .
this condition is more prevalent among women and typically occurs in the third decade of life .
it normally manifests as persistent , isolated cervical adenopathy with a recurrence rate of 3% .
the identification of this condition is of high significance , given the risk of misdiagnosis with other disorders such as malignant lymphoma and extensive necrosis .
the patient was a 32-year - old female diagnosed with kikuchi - fujimoto disease via neck lymph node biopsy in august 2006 in the city of mashhad , iran .
the disease regressed with proper follow - up , although after eight years the patient was readmitted to the hospital with severe weight loss , high fever , and uncommon symptoms of generalized adenopathy in cervical , axillary and inguinal regions .
although kfd is an uncommon condition , it should be featured in the list of differential diagnoses of tender lymphadenopathy , especially lymphadenopathy localized to the cervical region .
kikuchi - fujimoto disease ( kfd ) , also known as histiocytic necrotising lymphadenitis ( hnl ) , was first reported in japan in 1972 . kikuchi and fujimoto simultaneously but independently described the histological and clinical features of this inflammatory disease of lymph nodes and termed it as  subacute cervical necrotizing lymphadenitis  .
this disease occurs in all races , although the majority of cases have been reported among asians ( 2 ) .
the etiology of kfd is unknown , although some believe that viral agents are the main causes of this condition .
kfd usually occurs as a persistent , isolated adenopathy and often appears in the neck area .
this disease has been reported in different age groups ( 9 - 40 years old ) and is more prevalent among women ( 3 ) .
definite diagnosis of kfd is dependent on the biopsy and histology of lymph nodes ( 4 , 5 ) .
this disease usually resolves within 1 - 3 months and recurrence and death are quite rare ( 6 ) .
the patient was a 32-year - old female , a native of sistan and baluchestan province , iran , with complaints of afternoon fever ( 38c ) , night sweats , severe weight loss and anorexia .
she was referred to the division of infectious diseases at shahid hashemi nejad hospital in mashhad , iran , in august 2006 .
a month before the admission , she had received three different doses of ceftriaxone and acetaminophen for her high fever ; given her non - responsiveness to the treatment , she was readmitted to the hospital .
the patient was previously healthy and newly married , with no prior history of diseases or drug consumption .
she was lethargic , though hemodynamically stable according to physical examinations , and her body mass index ( bmi ) was 17 kg / m at the admission time .
the significant observation was a mobile mass ( 2  1 cm ) with soft consistency behind the left sternocleidomastoid in left cervical , axillary and inguinal lymph nodes ; no redness or warmth was reported in the mass .
ear , nose , and throat checkups showed normal results and the patient had no complaints of abdominal pain , lower limb pain , or diarrhea .
in addition , no facial or body skin rash was reported and other findings were normal . in para - clinical evaluations , the results of chest radiograph , chest ct scan , electrocardiogram and echocardiography were normal . moreover , in pelvic and abdominal ultrasounds , no abnormalities were observed except for the enlargement of the spleen . in the patient s initial blood test , white blood cell ( wbc ) count of 2000  
cells / mm and the presence of 1% atypical lymphocytes with an increase in immature neutrophils ( bands ) , hemoglobin of 11.3   g / dl and a mild thrombocytopenia , as well as high alkaline phosphatase , high c - reactive protein , and an erythrocyte sedimentation rate ( esr ) of 48 were reported .
blood tests and examinations of urine and stool samples were all negative . the results of sputum acid - fast bacilli culture and antinuclear antibody test were negative , as well .
similarly , the results of autoimmune screening , as well as toxoplasma and cytomegalovirus tests were negative .
macroscopic examination of the lymph node capsule showed a creamy grey - colored mass with soft consistency , homogeneity , and a dimension of 0.5  1  2 cm . in the microscopic study ( at low magnification ) , partial effacement of the lymph node structure and pale patchy infiltrates were reported .
at the center of the lesion , an extensive coagulative necrosis composed of cellular debris and free of neutrophilic or eosinophilic infiltrates was observed ( figures 1 and 2 ) . finally , the kikuchi s disease was diagnosed based on the findings . considering the improved symptoms of the patient after two weeks of hospitalization , she was discharged from the hospital in good general health and received anti - inflammatory medications .
given the improvements in her symptoms , the medications were gradually discontinued and she started to be followed up on a monthly basis . during the 8-year follow - up of the patient , except for the mean sublingual temperature of 37.8c which was reported once or twice a year ( continuing for a couple of days ) , no other significant findings were reported .
the patient was in good general health during the follow - up and her examination results were normal .
the patient had two healthy pregnancies in 2010 and 2012 and was in a good general condition during her pregnancies and lactation . in august 2014 ,
the patient was referred to the hospital with high fever , night sweats , weight loss and anorexia ( bmi = 16
after a one - month delay , painful generalized lymphadenopathy in left cervical , axillary and inguinal lymph nodes with soft consistency and mobility was reported ; however , no redness or warmth was reported in the mass . in the second blood test , neither leukopenia nor high esr was observed . the results of chest radiography as well as those of abdominal and pelvic ultrasounds were normal .
considering the patient s unwillingness to undergo repeated biopsy , she underwent a corticosteroid regimen .
after three months of using prednisone , the patient s fever subsided and her lymphadenopathy was impalpable .
kfd , also known as hnl , is a self - limiting condition , causing lymphadenopathy .
this disease is most commonly observed in adults younger than 40 years of age , with a female predominance in previous studies ( 3 ) .
other less commonly reported presentations include leukopenia , atypical lymphocytes on peripheral smear , liver dysfunction , bone marrow involvement , fatigue , hepatosplenomegaly , and skin rash ( 2 ) .
although the disease is characterized by regional lymphadenopathy , few patients show generalized lymphadenopathy . in the present case
, there was simultaneous involvement of cervical lymph nodes with axillary and inguinal lymph nodes together with uncommon symptoms including leukopenia , severe weight loss and splenomegaly , indicating a generalized lymphoma which often leads to a misdiagnosis .
a definite diagnosis of kfd may be made only via histopathological analysis by open lymph node biopsy ( 1 ) .
there is no specific treatment for kfd , although in severe cases , the use of corticosteroids has been recommended for the prevention of fatal complications ( 1 ) .
the signs and symptoms of kfd usually resolve after several months ( 1 , 2 ) . in a review of cases with kfd by kucukardali et al .
in addition , a low recurrence rate ( 3 - 4% ) has been reported in previous studies ( 1 , 2 ) .
patients with recurrent episodes are more likely to present fever , cough , and fatigue , along with frequent extranodal involvement in the initial presentation ( 7 ) . in comparison with previous studies ,
her disease was complicated by the return of symptoms eight years after the initial diagnosis , with symptoms such as fever , fatigue , severe weight loss and generalized lymphadenopathy . in the present case ,
a definite diagnosis of kfd is made by tissue biopsy , particularly whole lymph node biopsy ( 4 , 5 ) . in the present case ,
the diagnosis of hnl was confirmed according to the results from the pathological slices ; also , there is a chance that more severe cases get included with pathological confirmation , while mild cases get excluded due to follow - up loss .
although the etiology of kfd recurrence is unknown , certain viral infections including epstein - barr ( eb ) virus , parvovirus b19 and human herpesvirus-8 have been hypothesized to be among the triggers for kfd relapse ( 8) . as stephan et al
. indicated , the recurrence of hnl is associated with the persistence of eb virus infections ( 9 ) . in addition , atarashi et al . , reported a case of recurrent hnl in a human t - lymphotropic virus type i carrier . in the present case , infectious etiologies including eb virus , cytomegalovirus and hiv were all negative ( 10 ) .
it is unknown whether other viral infections were associated with hnl in the present case .
as evidence suggests , kfd may be a precursor for systemic lupus erythematosus ( sle ) , as both conditions have concurrent , coexisting patterns in patients ( 11 ) .
cheng et al . , described the clinical manifestations and outcomes of 195 patients diagnosed with hnl . in the mentioned study ,
a total of 14 out of 96 patients ( 14.6% ) had recurrent hnl and five of them developed autoimmune diseases such as sle ( 12 ) .
it seems that individuals with hnl are more susceptible to sle ; thus , they should be routinely screened for this disorder ( 2 ) . with the results of the present study ,
it is important to differentiate kfd from other conditions ; in this case , it can inhibit adverse drug reactions in patients and prevent empirical treatments , especially with antibiotics that impose financial burdens on families . in conclusion , although kfd is an uncommon condition , it should be featured in the list of differential diagnoses of tender lymphadenopathy , especially lymphadenopathy localized to the cervical region .
however , although the disease takes a self - limiting clinical course in most cases , we reported a case of kfd with a prolonged relapse of eight years . full recovery with a good response to corticosteroid regimen was achieved after the recurrence ; therefore , considering the recurrence of kfd , long - term follow - up of patients with kfd is necessary .
 introduction : kikuchi - fujimoto disease ( kfd ) is a benign , self - limited , inflammatory disorder , first reported in japan . 
 this condition is more prevalent among women and typically occurs in the third decade of life . 
 it normally manifests as persistent , isolated cervical adenopathy with a recurrence rate of 3% . 
 the identification of this condition is of high significance , given the risk of misdiagnosis with other disorders such as malignant lymphoma and extensive necrosis.case presentation : the patient was a 32-year - old female diagnosed with kikuchi - fujimoto disease via neck lymph node biopsy in august 2006 in the city of mashhad , iran . 
 the disease regressed with proper follow - up , although after eight years the patient was readmitted to the hospital with severe weight loss , high fever , and uncommon symptoms of generalized adenopathy in cervical , axillary and inguinal regions.conclusions:although kfd is an uncommon condition , it should be featured in the list of differential diagnoses of tender lymphadenopathy , especially lymphadenopathy localized to the cervical region . 
 we reported a case of kfd with a prolonged relapse of eight years . 
 full recovery with a good response to corticosteroid regimen was achieved after the recurrence . 
Introduction:
Case Presentation:
Conclusions:
1. Introduction
2. Case Presentation
3. Discussion