Kikuchi-Fujimoto disease


Also known as histiocytic necrotizing lymphadenitis.

Seen in young Asian individuals.

Clinical presentation includes fevers, lymphadenopathy and leukopenia, respiratory symptoms, arthralgias, myalgias, skin manifestations including ulcers of the skin with erythematous plaques and papules.

Kikuchi disease also known as Kikuchi-Fujimoto disease, and Histiocytic necrotizing lymphadenitis.

A rare, self-limiting disorder that typically affects the cervical lymph nodes.


Symptoms resolve within 1 to 4 months in most cases. 

Likely caused by a viral or an autoimmune process.

Viruses such as Epstein-Barr, HIV, herpes simplex, parvovirus B 19, and cytomegalic virus have been associated with this process.

Confused with tuberculosis, lymphoma, or even adenocarcinoma.

Mainly seen in Japan, with Isolated cases are reported in America, Europe and Asia.

Mainly a disease of young adults.

Mean age, 20–30 years, with a slight bias towards females.

The cause of this disease is not known.

Infectious and autoimmune etiologies have been proposed.

Sometimes associated with systemic lupus erythematosus.

Course of the disease is generally benign and self-limiting with lymphadenopathy most often resolving over several weeks to six months.

Recurrence rate is about 3%.

Mortality is extremely rare and usually due to hepatic, respiratory, or cardiac failure.

Some studies suggest a genetic predisposition to the proposed autoimmune response.

Several infectious diseases have been associated with Kikuchi’s disease.

Etiology obscure, but microbial/viral or autoimmune causes have been suggested.

But serologic tests including antibodies to a host of viruses have consistently proven noncontributory and no viral particles have been identified ultrastructurally.

Suspected to be a nonspecific hyperimmune reaction to a variety of infectious, chemical, physical and neoplastic agents.

SLE and other autoimmune conditions and manifestations such as antiphospholipid syndrome, polymyositis, systemic juvenile idiopathic arthritis, bilateral uveitis, arthritis and cutaneous necrotizing vasculitis have been linked to this disease.

May represent an exuberant T-cell mediated immune response in a genetically susceptible individual to a variety of non-specific stimuli.

Human leukocyte antigen class II genes are more frequent, suggesting a genetic predisposition to the proposed autoimmune response.

The signs and symptoms are fever, lymphadenopathy, skin rashes and headache.

Rarely hepatosplenomegaly and nervous system involvement resembling meningitis is seen.

Differential diagnosis includes SLE, disseminated tuberculosis, lymphoma, Sarcoidosis, and viral lymphadenitis, atypical infections and metastatic cancer

Sometimes IgM/IgG/IgA antibodies are present.

It is diagnosed by lymph node biopsy.

A self-limiting illness with no specific cure.

Treatment is supportive.

NSAIDs are used for tender lymph nodes and fever.

Corticosteroids are useful in severe extranodal or generalized disease.

Adenopathy is usually cervical.

Leukopenia is seen in 58% of cases.

Fine needle aspirate inadequate for diagnosis.

Generally resolves without treatment in less than six months.

Glucocorticoids and antibiotics such as ciprofloxacin and minocycline, may result in more rapid improvement.

Recurrence is rare.

Death due to the disease occurs 2% of patients.

Diagnostic test requires excisional biopsy.

Biopsy shows eosinophilic areas with necrotic and apoptotic debris, mixed with small lymphocytes, immunoblasts, and phagocytic histiocytes.

Phagocytic histiocytes contain eosinophilic proteinaceous debris with eccentric crescent nuclei.

Immuno histological staining for CD 123 confirms the presence of plasmacytoid dendritic cells, a feature of this disease.

Differential diagnosis includes lymphoma, systemic lupus related lymphadenitis, and infectious lymphadenopathy.

Diagnosis can only be determined through open biopsy of the affected lymph nodes.

Biopsy shows necrotizing lymphadenitis with a paucity of neutrophils, eosinophils, plasma cells, and granulomas with an abundance of cresentric histiocytes.

Microscopically shows alteration in the normal lymph node architecture, with cortical and paracortical foci with necrosis and a histiocytic cellular infiltrate.

Biopsy also may show coagulative necrosis and abundant eosinophilia apoptotic karyorrheic debris is also seen.

Pathologic diagnosis requires the presence of crescent-shaped histiocytes and plasmacytoid monocytes with scattered karyorrhexis.

Some patients who have an initial diagnosis of K-F disease actually had an early form of SLE.

Difficult to distinguish between SLE on biopsy and also peripheral T-cell lymphomas.

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