Saturday 30 October 2010

Kikuchi's Disease (Kikuchi-Fujimoto Disease or Histiocytic Necrotizing Lymphadenitis)

Kikuchi's Disease General Facts:
  • Also known as histiocytic necrotizing lymphadenitis & Kikuchi-Fujimoto disease.
  • Clinically non-cancerous enlargement of the lymph nodes.
  • First described in Japan by Dr Masahiro Kikuchi in 1972, & also independently by Y. Fujimoto.
Epidemiology:
  • Very rare disease mainly seen in Japan.
  • There have been isolated cases are reported in America, Europe & Asia.
  • Mainly a disease of young adults (mean age, 20–30 years).
  • Female preponderance.
Aetiology:
  • Cause of this disease is unknown.
  • Proposed infectious & autoimmune causes.
  • Possible genetic predisposition.
  • Suspected infectious candidates include - CMV, EBV, HSV, Varicella zoster virus, parainfluenza virus, parvovirus B19, paramyxovirus.
Clinical:
  • Clinical features include - fever, lymphadenopathy, skin rashes and headache.
  • Occasionally there may be features of hepatosplenomegaly or meningitis.
  • Laboratory findings include leucopenia, atypical lymphocytes & raised ESR.
  • Course of the disease is generally benign & self-limiting.
  • Lymphadenopathy most often resolves over weeks to 6 months.
  • Around 3% recurrence rate.
  • Death is very rare - from hepatic, respiratory, or cardiac failure.
Diagnosis:
  • Based on histology of lymph node showing necrotizing lymphadenitis.
Differential Diagnosis:
  • Other causes of lymphadenopathy
  • Cat-scratch fever
  • CMV infection
  • Disseminated tuberculosis
  • Fungal infections
  • HIV infection
  • Lymphoma
  • Sarcoidosis
  • SLE
  • Toxoplasmosis
  • Viral lymphadenitis
Tags: Histiocytic Necrotizing Lymphadenitis - Kikuchi's Disease - Kikuchi-Fujimoto Disease
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