In the latest Case Record of the Massachusetts General Hospital, a 30-year-old man was admitted to the hospital because of fever, chills, and lymphadenopathy that developed 6 months after he returned from a trip to India. Imaging revealed diffuse lymphadenopathy in the neck and chest and splenomegaly. Diagnostic tests were performed.
Localized lymphadenopathy is common, and 55% of all cases involve lymph nodes in the head and neck, including the cervical region. In contrast, 1% of all cases of lymphadenopathy are attributed to supraclavicular lymph nodes, 5% to axillary nodes, and 14% to inguinal nodes.
• What is Kimura’s disease?
Kimura’s disease, which is often confused with angiolymphoid hyperplasia with eosinophilia, is a rare, benign, chronic inflammatory disorder of unknown cause that mainly affects young Asian men. Patients with Kimura’s disease have an indolent presentation, with painless lymphadenopathy in the head and neck. It is common to see eosinophilic infiltrates in the biopsy specimens.
• What is Kikuchi-Fujimoto disease?
Kikuchi-Fujimoto disease, which was first described in 1972, is seen in young Asian persons. The clinical features include symptom onset over a period of 2 to 4 weeks, fevers, lymphadenopathy (usually cervical), leukopenia (in less than or equal to 58% of cases, according to the original description of the disease by Kikuchi), respiratory symptoms, arthralgias and myalgias, and skin manifestations (e.g., ulcers, erythematous plaques, and papules). Fine-needle aspiration biopsy of a lymph node is not a reliable diagnostic test for Kikuchi-Fujimoto disease; the aspirate usually shows crescentic histiocytes, karyorrhectic and granular debris, and plasmacytoid monocytes.
Morning Report Questions
Q: What is the prognosis of Kikuchi-Fujimoto disease?
A: Generally, Kikuchi-Fujimoto disease resolves without treatment in less than 6 months, although the use of glucocorticoids and such antibiotics as ciprofloxacin and minocycline may result in improvement. Recurrences have been reported but are rare, regardless of treatment modality; death due to the disease is also rare, occurring in approximately 2% of patients.
Q: What condition is histologically closest to Kikuchi-Fujimoto disease, leading to possible misdiagnosis?
A: SLE [systemic lupus erythematosus]-related lymphadenitis is the condition histologically closest to Kikuchi-Fujimoto disease. Hematoxylin bodies (basophilic condensations of nuclear and cytoplasmic debris) and the Azzopardi effect (which occurs when nuclear debris adheres to the surface of blood vessels) are findings specific to SLE-related lymphadenitis, but they are uncommon. In patients with SLE-related lymphadenitis, the lymph nodes tend to contain more neutrophils and plasma cells than do those in patients with Kikuchi-Fujimoto disease; the presence of vasculitis outside necrotic areas is also more common in patients with SLE-related lymphadenitis. SLE may occasionally develop in patients months or years after the findings on a lymph-node biopsy suggested Kikuchi-Fujimoto disease.