Abstract
Kikuchi-Fujimoto Disease (KFD) is a benign, self-limiting entity seen in Asia with only a few reports in the United States. Patients present with a triad of cervical lymphadenopathy, headache, and fever. Laboratory and imaging findings are nonspecific. Diagnosis is made on histopathological examination of the affected lymph nodes. This disease mimics infectious, autoimmune, and malignant processes. It is important to include this on the differential diagnosis for cervical lymphadenopathy because treatment and prognosis differ. We present a case of KFD in a 34 year old Guatemalan female who presented with cervical lymphadenopathy, headache, and fever, and a review of the current literature.
Introduction
A rare cause of cervical lymphadenopathy and fever infrequently reported in the United States is Kikuchi-Fujimoto Disease (KFD). It is a benign, self-limiting process that primarily affects women under 40 and is more commonly seen in those of Asian origin [ ]. The etiology of KFD is unknown and laboratory and imaging tests are usually unremarkable or unreliable for diagnosis [ , ]. Definitive diagnosis must be obtained by excisional biopsy and histopathological examination of the tissue [ , ]. This review of the literature is informed by a case report of a 34-year-old Guatemalan female who presented with tender cervical lymphadenopathy, headache, and fever.
Case report
A 34-year-old Guatemalan female presented with a 1-week history of left neck pain and swelling, severe headaches, otalgia, fatigue, fevers of 38.3 °C (101.0 °F), and night sweats. She emigrated from Guatemala at 19 years old but had no recent history of travel or sick contacts. Relevant medical history included positive purified protein derivative (PPD) treated with a course of isoniazid years prior. Physical exam revealed firm, tender, mobile cervical lymphadenopathy at levels 2, 3, and 5. Laboratory findings were a mild leukopenia without anemia, thrombocytopenia, or liver dysfunction. Autoimmune panel (ANA IgG, RF, anti-CCP Ab IgG, anti-dsDNA, C3/C4 levels, anti-SSA, p-ANCA, and c-ANCA) and evaluation for infection (EBV and HIV) were negative. Chest x-ray and repeat PPD were negative.
Neck computed tomography (CT) with intravenous contrast showed hyperenhancing lymphadenopathy of left levels 2, 3, 4 and left supraclavicular nodes in addition to non-enlarged hyperenhancing left level 5 lymph nodes ( Fig. 1 A, B, 1C). The largest node present was 2.9 cm in diameter. An excisional biopsy of left level 2 node grossly revealed gray, friable, enlarged lymph nodes, with histologic analysis illustrating paracortical areas of sterile necrosis with abundant histiocytes and karyorrhectic debris ( Fig. 2 A and B). Additional assessments of the excised lymph node (T-cell receptor gamma gene rearrangement by polymerase chain reaction (PCR), flow cytometry, immunohistological staining, acid-fast bacilli staining) were unremarkable. In the absence of laboratory abnormalities, this histologic pattern is consistent with KFD. Two months after initial presentation, the patient reported complete resolution of all symptoms.
