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
Posted by Medicalchemy
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