A 19-year old Caucasian female with a history of atopic dermatitis and mild asthma developed an erythematous maculopapular rash on her bilateral palms and wrists, right-sided tender cervical lymphadenopathy, and nightly fevers and headaches five days after returning from a one-month trip to Cambodia. She presented two weeks after returning from her trip due to persistent symptoms, though her rash had mostly resolved. On admission her WBC count was 6.06 with increased atypical lymphocytes and hemoglobin of 10.7. She continued to have nightly headaches, drenching night sweats, and fevers to 38.8°C. Differential was broad given fever and related symptoms in a returned traveler. Pertinent negatives included HIV 1/2, HTLV 1/2, EBV, CMV, quantiferon gold TB, multiple thick and thin blood smears, and blood cultures with each fever. Dengue IgG/IgM, Chikungunya IgG/IgM, Leptospira IgM, Strongyloides IgG, Zika PCR, rapid plasma reagin (RPR), Lyme ELISA and Western blot analyses, stool O&P, and ANA were also negative. Serum IgE level was elevated at 2732. Lumbar puncture revealed aseptic meningitis (opening pressure 25.5 mmH2O, 8 WBCs with 94% lymphocytes in tube 1, 6 WBCs with 91% lymphocytes in tube 4, normal glucose and protein). She was started on empiric doxycycline on HD4 for possible Rickettsial disease. On HD5, core biopsy and FNA were performed on the largest anterior cervical lymph node. Her fever curve gradually improved and she was discharged on HD6 to complete a course of doxycycline. Following discharge, lymph node biopsy results were compatible with Kikuchi’s lymphadenitis. The doxycycline was discontinued. Her symptoms were resolved at clinic follow-up one week later.
Discussion:
Kikuchi-Fujimoto Disease (KFD), or histiocytic necrotizing lymphadenitis, is a rare, self-limiting disease of unknown etiology occurring mostly in young women and in Asia. It usually presents as a mild febrile illness with benign cervical lymphadenopathy. Our patient also demonstrated less commonly associated rash, headache, night sweats, and aseptic meningitis. A retrospective study by Cheng et al. of 195 patients with KFD found that headache occurred in 16.9%, night sweats in 5.6%, and rash in only 4.1%. Aseptic meningitis is such a rare presentation for KFD that it can confuse workups (Park et al., 2009). Furthermore, though most likely an incidental finding in our patient given her known atopy, elevated IgE levels can occur with eosinophilia in Kimura Disease, another rare cause of benign lymphadenopathy.
Conclusions:
KFD should be considered in all patients with fever and lymphadenopathy, even in the setting of recent travel. Suspicion should be increased in young women with mild disease and typical symptoms, but atypical symptoms such as rash, night sweats, or aseptic meningitis should also raise suspicion in the appropriate clinical setting. Early lymph node biopsy is essential.