An uncommon presentation of Kikuchi Fujimoto disease: a case report with literature review

Abstract

BackgroundKikuchi–Fujimoto disease is so named because Kikuchi and Fujimoto were the first scientists to describe it in Japan in 1972. Although the disease has been reported from all over the world and more so from Asia, it is rare. To date only eight cases have been reported from Nepal. Cervical lymphadenopathy, fever and raised Erythrocyte Sedimentation Rate are usual presenting features of this disease. We describe a case which presented with thrombocytopenia and axillary lymphadenopathy in addition to the usual features. Out of the total eight cases that have been reported from Nepal so far, no patients had thrombocytopenia and only one patient had axillary lymphadenopathy.Case presentationA 24-year-old Nepali female presented with a 3-week history of low-grade fever, headache, and painful, discrete, unilateral left-sided cervical and axillary lymphadenopathy. Among the multitude of tests that were carried out, Erythrocyte Sedimentation Rate was raised and there was thrombocytopenia while other tests were normal. Painful lymphadenopathy pointed to bacterial lymphadenitis while chronic low-grade fever suggested tuberculosis. A cervical lymph node was excised for histopathological examination to reach an accurate diagnosis. On the basis of pathognomonic features viz., paracortical foci composed of various types of histiocytes including crescentic type in the background of abundant apoptotic karyorrhectic debris, a diagnosis of Kikuchi–Fujimoto disease was made. On follow-up evaluation after 6 weeks, the patient had no systemic symptoms, enlarged lymph nodes had regressed in size significantly, and Erythrocyte Sedimentation Rate and platelet count had become normal.ConclusionKikuchi–Fujimoto disease should be kept in the differential diagnosis of lymphadenopathy in young patients, female or male even in tuberculosis-endemic countries and even in patients who have unusual features; for example thrombocytopenia and involvement of axillary lymph nodes in addition to cervical lymph nodes as in this case.

Kikuchi-Fujimoto disease in the regional lymph nodes with node metastasis in a patient with tongue cancer: A case report and literature review

“…Kwon et al ( 32 ) reported that the mean maximum diameter of the affected cervical LNs was 1.62 cm in 1,196 KFD LNs from 96 patients ( 32 ). Furthermore, KFD LNs may be >2.5 cm ( 4 , 43 ). KFD LNs may thus resemble metastatic lesions, based on palpation, though clinical palpation of the neck has been reported to be unreliable ( 44 ).…”

“…Youk et al ( 48 ) also indicated that KFD tended to be misdiagnosed as malignancy on the basis of US findings. Furthermore, FDG uptake in FDG-PET/CT is not specific for cancer ( 4 , 47 ). Regarding time to recurrence, KFD typically recurs within 1 year of its initial resolution ( 5 , 49 ), while LN metastasis also occurs within 1 year ( 50 ).…”

“…Up until 2013, 733 patients with KFD have been reported ( 3 ). Most patients with KFD present with localized unilateral cervical lymphadenopathy (axillary and inguinal are infrequently) with or without pain and fever ( 4 ). KFD occurs worldwide ( 5 ) with a relatively high prevalence, particularly among young women and girls in Japan, as well as in other Asian countries ( 6 ).…”

Kikuchi Fujimoto disease: A rare case of recurrence

“…Kikuchi-Fujimoto Disease (KFD) is the most common cause of histiocytic necrotizing lymphadenitis (non-granulomatous inflammation and necrosis of the lymph node), followed by systemic lupus erythematosus -SLE and acute Epstein Barr virus -EBV lymphadenitis [1,2]. It is a rare disease that was described for the first time in Japan, independently by Kikuchi and Fujimoto, in 1972 [1,2].…”

“…Kikuchi-Fujimoto Disease (KFD) is the most common cause of histiocytic necrotizing lymphadenitis (non-granulomatous inflammation and necrosis of the lymph node), followed by systemic lupus erythematosus -SLE and acute Epstein Barr virus -EBV lymphadenitis [1,2]. It is a rare disease that was described for the first time in Japan, independently by Kikuchi and Fujimoto, in 1972 [1,2]. It is a benign and self-limiting condition characterized by regional lymphadenopathy (usually posterior cervical, in 60-90% of cases) associated with fever, and other signs and symptoms such as cutaneous lesions, arthritis, fatigue and hepatosplenomegaly [3,4].…”