What is Kikuchi disease?

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

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