What is Kikuchi disease?

Updated: Nov 13, 2020
  • Author: John Boone, MD; Chief Editor: Sara J Grethlein, MD, FACP  more...
  • Print
Answer

Kikuchi disease, also called histiocytic necrotizing lymphadenitis or Kikuchi-Fujimoto disease, is an uncommon, idiopathic, generally self-limited cause of lymphadenitis. Kikuchi first described the disease in 1972 in Japan. Fujimoto and colleagues independently described Kikuchi disease in the same year. [2]

The most common clinical manifestation of Kikuchi disease is cervical lymphadenopathy, with or without systemic signs and symptoms. [3, 4, 5, 6] Clinically and histologically, the disease can be mistaken for lymphoma or systemic lupus erythematosus (SLE). [2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17]

Kikuchi disease almost always runs a benign course and resolves in several weeks to months. Disease recurrence is unusual, and fatalities are rare, although they have been reported. [2, 18]

In a retrospective study of 24 cases of Kikuchi disease in southern India over a 4-year period, the findings included the following [19] :

  • Sex - 17  females, 7 males
  • Most common presenting feature - Painless cervical lymphadenopathy
  • Most common laboratory abnormalities - Leukopenia, increased erythrocyte sedimentation rate (ESR), increased liver enzyme levels
  • Lymph node biopsy findings - Typical morphology of Kikuchi disease, with necrosis, karyorrhectic debris, and the presence of the typical cell types, namely crescentic histiocytes and plasmacytoid monocytes
  • Clinical resolution - Within 1 month

Treatment of Kikuchi disease is generally supportive. (See Treatment.) Pharmacologic therapy that has been recommended includes the following:

  • Nonsteroidal anti-inflammatory drugs (NSAIDs) - To alleviate lymph node tenderness and fever
  • Corticosteroids  - For severe extranodal or generalized Kikuchi disease
  • Hydroxychloroquine - For steroid-resistant or recurrent cases
  • Intravenous immunoglobulin - For steroid-resistant or recurrent cases

See also Cutaneous Kikuchi Disease

For patient education resources, see Swollen Lymph Nodes and Lymphoma.


Did this answer your question?
Additional feedback? (Optional)
Thank you for your feedback!