A 29-year-old healthy woman presented to the emergency department with complaints of fever, chills and malaise for one week. She had been taking ibuprofen at home with no relief. She also noticed swelling in her neck bilaterally. There was no history of recent travel, sick contacts or exposure to people with tuberculosis. She denied dysuria, cough, diarrhea, shortness of breath, chest pain, menorraghia, night sweats or weight loss. She has been in a monogamous relationship for many years. There was no family history of lymphoma, leukemia, systemic lupus erythematosus, rheumatoid arthritis or any other rheumatologic disorders. She did not take any prescription or herbal medications. Social history was negative. Vital signs in the emergency department revealed a temperature of 103.2 F and a pulse of 113. Physical exam was normal except for bilateral anterior and posterior cervical, pre and post-auricular, submandibular, submental and right axillary lymphadenopathy. Initial laboratory studies revealed white blood cell count-3000 th/mm3, hemoglobin-8.8 g/dL, platelets-273 th/mm3 and mean corpuscular volume-66.2 mcm3. Chest x-ray was normal. Subsequently, she was admitted to the inpatient floor with persistent fevers and lymphadenopathy. Further laboratory studies revealed anemia of chronic disease. Hemolytic work-up was negative. Hemoglobin electrophoresis revealed no evidence of beta thalessemia. HIV, EBV monospot, Toxoplasma, and CMV serologies were negative. The patient continued to experience high fevers with rapidly increasing diffuse lymphadenopathy and further laboratory testing was ordered. Peripheral blood flow cytometry revealed no monoclonal B cell population that was identified and no evidence of paroxysmal nocturnal hemoglobinuria. The throat culture revealed beta hemolytic streptococcus and she was prescribed penicillin. CT scans of the chest, and abdomen/pelvis with intravenous contrast revealed enlarged bilateral axillary lymphadenopathy measuring up to 2.7 cm in largest dimensions. CT scan of the neck with intravenous contrast showed diffuse enhancing lymphadenopathy involving all neck levels, soft tissues and axillae bilaterally. Surgery consultation was obtained for the consideration of an excision lymph node biopsy. Further laboratory studies revealed an elevated IgG level. Antinuclear antibody and anti-double stranded DNA testing were negative. beta2 microglobulin level was elevated. Angiotensin-converting enzyme level was normal. She underwent excisional lymph node biopsy of the left cervical lymph node and was discharged home with instructions to take acetaminophen if she had recurrent fevers. The pathology report of the lymph node revealed lymphoid tissue with extensive geographic necrosis (necrotizing histiocytic inflammation) suggestive of Kikuchi's disease (Fig. 1). There was no evidence of infection in the lymph node specimen. The patient was informed of the diagnosis and told to return to the outpatient clinic for follow-up evaluation.
One month later, the patient presented to the outpatient clinic. The fevers had resolved and the lymph nodes had markedly reduced in size to less than a centimeter clinically. The hemoglobin was improving. She had further work-up to search for an identifiable cause of her condition. Serologic testing for human herpes virus 6, human herpes virus 8, and respiratory virus panel were negative except for an elevated parvovirus IgG level. Repeat HIV testing was also negative. She was also referred to the rheumatology clinic because she began experiencing morning stiffness. On a three month follow-up visit, she continued to be afebrile and the lymphadenopathy and microcytic anemia had resolved. Although the microcytic anemia had resolved, she was recommended to undergo a colonoscopy and upper endoscopy to evaluate the etiology of the microcytic anemia. Laboratory testing done in the rheumatology clinic revealed no evidence of any rheumatologic conditions.