A 35-year-old African-American woman presented to the Tampa General Hospital emergency room with 3 days of pain in her ankles, wrists, neck, and back. She also complained of increasing neck stiffness and fever with shaking chills. She was in good health until 1 month prior, when she was admitted to the hospital with right facial cellulitis and a periodontal abscess confirmed by computed tomographic scan. The patient was treated with oxacillin and clindamycin in the hospital. She was sent home on oral clindamycin but had not been taking this regularly. She had 2 episodes of nonbloody nonbilious emesis on the day of admission. No family members or friends have had similar symptoms. She smoked 5 cigarettes per day and denied alcohol use. She had a history of polysubstance abuse but denied intravenous drug use. She was divorced and living with her boyfriend. The patient denied any sexually transmitted disease and reported a negative HIV test 1 year ago. She had been taking ibuprofen and oxycodone with acetaminophen as needed for periodontal pain.
On physical examination, she was alert and oriented with a temperature of 103°F, pulse of 102 beats per minute, respiratory rate of 22 breaths per minute, and blood pressure of 102/67 mm Hg. Pertinent physical findings included a 2/6 systolic ejection murmur at the left lower sternal border. All other findings were within normal limits. Her neck was supple and nontender, with no cervical, supraclavicular, or submandibular lymphadenopathy. Kernig and Brudzinski signs were negative.
A complete blood count with differential, complete metabolic profile, and urinalysis were within normal limits. A lumbar puncture disclosed a normal opening pressure, negative Gram stain and culture, no cells, and normal protein and glucose concentrations. Cerebrospinal fluid India ink stain and cryptococcal antigen were negative. Cerebrospinal fluid and sputum acid-fast bacillus smears were negative. Blood and sputum cultures were negative. A slide test for infectious mononucleosis heterophile antibodies was negative. The chest roentgenogram was clear without infiltrates, and no abnormalities were seen on electrocardiogram. Computed tomographic scan of the head was unremarkable. Computed tomographic scan of the neck showed resolution of the inflammatory changes adjacent to the right side of the mandible as compared with the previous study. Small subcentimeter lymph nodes remained in the submandibular region.
The patient was initially started on ceftriaxone, vancomycin, and ampicillin for empiric bacterial meningitis treatment. When cerebrospinal fluid results returned negative, ampicillin and vancomycin were stopped; clindamycin and fluconazole were started. Throughout the hospitalization, the patient continued to have fever spikes. Five sets of blood cultures were negative. On the fourth hospital day, clindamycin and ceftriaxone were stopped, and piperacillin-tazobactam was started. At that time, the patient developed a 3 × 4 cm, firm, smooth, discrete, and mobile mass in the left submandibular area. The nodal enlargement was tender to palpation. Otolaryngology was consulted for a biopsy of the mass. Repeat laboratory testing and serologies were negative. Thyroid-stimulating hormone and lactate dehydrogenase level were within normal limits. HIV, antinuclear antibody, rapid plasma reagin test, cytomegalovirus polymerase chain reaction, Epstein-Barr virus polymerase chain reaction, and Bartonella titers were all negative. A 2-dimensional echocardiogram revealed a normal ejection fraction with mitral valve prolapse but showed no valvular vegetations. A left cervical open lymph node biopsy was performed. The pathology report revealed necrotizing lymphadenitis with areas of histiocytic proliferation (Figs. 1 and 2). The lymph node flow cytometry showed no abnormality of lymphoid populations, and no distinct monoclonal population was identified.
At this point, our management changed. All antibiotics and antifungals were stopped. Prednisone was started at 40 mg twice daily. Lymph nodes reduced in size within 48 hours. Fevers, myalgias, and arthralgias resolved, and the lymphadenopathy markedly decreased in size over 48 hours. The patient was discharged to home to be followed up in the clinic in 2 weeks. Two months later, the patient remained asymptomatic with no palpable lymph nodes or fever.
Kikuchi-Fujimoto disease usually presents as a febrile illness with unilateral enlarged cervical lymphadenopathy of less than 2 cm.2 This localized lymphadenopathy is frequently accompanied by fever and flulike symptoms and, on rare occasions, can also present with weight loss, nausea, and vomiting. Diagnosis is made by lymph node excision biopsy, which reveals patchy paracortical areas of necrosis surrounded by histiocytes with crescentic nuclei, plasmacytoid monocytes, immunocytes, karyorrhectic debris, and an absence of neutrophils.3 The differential diagnosis of necrotizing lymphadenitis includes lymphoma, systemic lupus erythematosis, and various infections.4 However, the particular histological findings associated with this case are virtually pathognomonic for Kikuchi disease. Although it lacks a clear etiology, Kikuchi disease has been reportedly associated with infections such as Epstein-Barr virus, rubella, paramyxovirus, parainfluenza virus, Yersinia, and Toxoplasma.3 The disease typically runs a self-limiting course with spontaneous resolution occurring anywhere from 1 to 6 months after the diagnosis. In some cases, glucocorticoid therapy has been used to shorten the course of the disease or for treatment of recurrent disease.5
This case is of particular interest because it masqueraded as a complication from a periodontal abscess. It was also unusual in both the delay of lymph node enlargement from the initial symptoms and the larger-than-usual lymph node. Prednisone did result in improvement in symptoms. Physicians should be aware of Kikuchi-Fujimoto disease and include it in the differential of cervical lymphadenitis.
1. Spies J, Foucar K, Thompson C, et al. The histopathology of cutaneous lesions of Kikuchi's disease (necrotizing lymphadenitis). Am J Surg Pathol
2. Swartz MN. Lymphadenitis and lymphangitis. In: Mandell. Principles and Practice of Infectious Diseases
. 5th ed. Orlando, Fla: Churchill Livingstone, Inc; 2000;1071.
3. Hsin-Ching L, Chih-ying S, Chao-Cheng H, et al. Kikuchi's disease: a review and analysis of 61 cases. Otolaryngol Head Neck Surg
. 2003; 128(9):650-653.
4. Onciu M, Medeiros J. Kikuchi-Fujimoto lymphadenitis. Adv Anat Pathol
5. Jang YJ, Hwan K, Seok HJ. Management of Kikuchi's disease using glucocorticoid. J Laryngol Otol