We report this case of a 33-year-old African American woman who presented to the clinic with preauricular and submandibular masses that she had noticed 6 weeks earlier. She gave a remote history of noticing bilateral cervical masses 3 years prior to this presentation that had not been investigated at the time and resolved spontaneously. Excisional biopsies of the cervical lymph nodes showed morphologic and immunophenotypic findings suggestive of Kikuchi Fujimoto disease (KFD). KFD is an uncommon, self-limited, and perhaps an underdiagnosed entity with an excellent prognosis. It mimics malignant lymphoma in presentation and therefore an accurate clinicopathological differentiation is crucial.
Cervical lymphadenopathy could be a manifestation of a variegated group of illnesses ranging from benign infectious causes to malignant lymphomas. Kikuchi-Fujimoto disease (KFD), also called histiocytic necrotizing lymphadenitis, was initially described independently by Kikuchi and Fujimoto in 1972. It is a rare, benign, and self-limited syndrome of unknown etiology characterized by tender localized lymphadenopathy, constitutional symptoms such as fever and night sweats [
A 33-year-old African American woman with no medical comorbidities and a recent trip to Jamaica presented to the clinic with complaints of multiple right-sided neck swellings. She reported developing laryngitis while she was in Jamaica, following which she noticed a right preauricular and a right submandibular mass that progressively increased in size. She also complained of fatigue, malaise, and intermittent chills. She had an episode of bilateral cervical lymphadenopathy 3 years earlier that spontaneously resolved. She had no prior history of tuberculosis or contact with it. She denied any history of smoking tobacco, drinking alcohol, or using illicit drugs. She denied taking any herbal or nonprescription medications at her house.
Physical examination revealed a comfortable and well-built young woman with anterior cervical, preauricular, submental, and right supraclavicular lymphadenopathy. The lymph nodes were firm and nontender. There was no hepatosplenomegaly, scleral icterus, or clinically appreciable lymphadenopathy elsewhere. Laboratory studies were significant for leucopenia with a total leucocyte count of 3200/
Chest X-ray was unremarkable. As the presentation was suspicious of lymphoma, a CT (computerized tomography) scan of neck, chest, abdomen, and pelvis was performed. It revealed asymmetric lymphadenopathy in the neck involving lymph nodes within the right posterior triangle (size of 2.8 cm), right internal jugular chain (size of 1.5 cm), bilateral submental lymph nodes (size of 1.2 cm), and right supraclavicular chain (size of 2 cm) (Figure
CT scan showing anterior and posterior cervical lymphadenopathy.
Lymph node showing reactive germinal centers and expanded paracortical areas suggestive of Kikuchi Fujimoto disease.
Multiple areas showing necrotic foci with apoptotic debris.
Patient was treated symptomatically with decrease in size of the lymph nodes gradually over the successive few weeks. Patient also experienced improvement in her fatigue and malaise. ANC improved to 1100/
KFD was initially reported in 1972 by Japanese pathologists Kikuchi and Fujimoto et al. independently. They described the disease as “lymphadenitis with focal proliferation of reticular cells accompanied by numerous histiocytes and extensive nuclear debris.” The incidence of KFD is unknown. It typically occurs in patients during third and fourth decade of life and is 3 to 4 times more common in women than in men. The disease is more prevalent in Asian populations. This geographic predominance may be related to the presence of certain HLA alleles such as HLA class II alleles,
There is much speculation about the etiology of KFD. An infectious or autoimmune cause has been suggested. Infectious agents such as
The onset of KFD could be acute or subacute, evolving over two to three weeks. The main clinical feature of KFD is typically unilateral lymphadenopathy, with cervical involvement in 70% to 98% of cases [
A review of the literature suggests that neutropenia and an elevated erythrocyte sedimentation rate were the major abnormal hematological findings. A few patients had atypical lymphocytes in the peripheral smear [
This disorder does not have a characteristic radiological appearance [
The definitive diagnosis of KFD is made through lymph node excision biopsy and histologic examination [
The differential diagnosis of a slow-growing neck mass is extensive, including malignant lymphoma, SLE, Hodgkin disease, toxoplasmosis, metastatic carcinoma, infectious mononucleosis, acquired immunodeficiency syndrome, cat-scratch disease, and angioimmunoblastic lymphadenopathy [
Kikuchi-Fujimoto disease is typically a self-limited disease that rarely requires specific treatment and resolves within one to four months [
Our case was unique as it represented a relapse of KFD in a non-Asian woman. Our patient had transaminitis and granulocytopenia with neutropenia that improved as the size of lymph nodes decreased. This gives an insight into the natural course of the disease. KFD is an uncommon, self-limited, and perhaps an underdiagnosed condition with an excellent prognosis. Unfortunately, the etiology, pathogenesis, diagnosis, and management of KFD still remain enigmatic and further research is required to answer these questions.
All Authors have confirmed that the paper is not under consideration for review at any other journal.
The authors of the paper, do not have a direct financial relation with the commercial identities mentioned in the paper that might lead to a conflict of interests.
All Authors have made contributions to the paper and have reviewed it before submission.