Case Presentation:

A 29–year–old woman presented to the hospital with fever, chills, and bilateral neck swelling for 1 week. There was no history of recent travel, sick contacts or exposure to tuberculosis. On presentation vital signs revealed a temperature of 103.2 F and a pulse of 113. Physical exam was unremarkable except for bilateral symmetrical cervical, pre–auricular, post–auricular, submandibular, submental and axillary lymphadenopathy. Initial laboratory studies were normal except for the presence of microcytic anemia. Hemolytic work–up and hemoglobin electrophoresis were negative. HIV, EBV monospot, Toxoplasma and CMV serologies were negative. Peripheral blood flow cytometry revealed no monoclonal B cell population. CT scans of the neck, chest, and abdomen/pelvis with intravenous contrast revealed diffuse enhancing bilateral lymphadenopathy involving the neck and the axilla. Antinuclear antibody and anti–double stranded DNA were negative. She underwent excisional biopsy of the left cervical lymph node. The pathology revealed lymphoid tissue with necrotizing histiocytic inflammation. In the context of a negative ANA and flow cytometry, the results were suggestive of Kikuchi’s disease. On a one month follow–up visit, she was afebrile and had minimal residual lymphadenopathy. Serologic testing for human herpes virus 6, human herpes virus 8, and respiratory virus panel were negative except for an elevated parvovirus IgG level. On a 3 month follow–up visit, she continued to be afebrile and there was complete resolution of lymphadenopathy and microcytic anemia.

Discussion:

Kikuchi’s disease is a rare and benign condition that has been mainly described in women. It can mimic serious conditions like lymphoproliferative disorders, systemic lupus erthematosus and tuberculosis lymphadenitis. Presenting symptoms include fever and cervical lymphadenopathy. The pathogenesis is not clear. Lymph node biopsy is the definitive way to make the diagnosis. Necrotic foci can be seen on gross examination. Paracortical foci with histiocytic infiltrate are seen on microscopic examination. Treatment is generally supportive. The signs and symptoms typically resolve in 1 to 4 months. Kikuchi’s disease is self–limited but the patients should be followed for a few years since recurrence is common and some of the patients can develop systemic lupus erythematosus.

Conclusions:

Although Kikuchi’s disease is a rare entity, we should consider it in the differential diagnosis when a young woman presents with fever and cervical lymphadenopathy. Accurate diagnosis of this condition is essential in preventing unnecessary emotional and mental distress associated with labeling a patient as having a more serious condition such as lymphoma or systemic lupus erythematosus.