Kikuchi disease

Kikuchi disease
Classification and external resources
Micrograph of a lymph node with Kikuchi disease showing the characteristic features (abundant histiocytes, necrosis without neutrophils). H&E stain.
eMedicine	med/3663
MeSH	D020042

Kikuchi's disease, also known as histiocytic necrotizing lymphadenitis^[1] and Kikuchi-Fujimoto disease,^[2]^[3] is a rare, non-cancerous enlargement of the lymph nodes.

It was first described in Japan by Dr Masahiro Kikuchi in 1972^[4] and independently by Y. Fujimoto.

1 Epidemiology
2 Pathophysiology
3 Clinical Features
4 Diagnosis
5 Management
6 See also
7 References

Epidemiology

Kikuchi's disease is a very rare disease and mainly seen in Japan. Isolated cases are reported in America, Europe and Asia. It is mainly a disease of young adults (mean age, 20–30 years), with a slight preponderance towards females. The cause of this disease is not known although infectious and autoimmune etiologies have been proposed. Course of the disease is generally benign and self-limiting. Lymphadenopathy most often resolves over several weeks to six months. Recurrence rate is about 3%. Mortality is extremely rare and usually due to hepatic, respiratory, or cardiac failure.

Pathophysiology

Some studies have suggested a genetic predisposition to the proposed autoimmune response. Several infectious candidates have been associated with Kikuchi's disease,^[5] including cytomegalovirus, Epstein Barr virus, Herpes simplex virus, Varicella zoster virus, parainfluenza virus, parvovirus B19 and paramyxovirus. Association between systemic lupus erythematosus (SLE) and Kikuchi's disease is not proved.

Clinical Features

The signs and symptoms of Kikuchi's disease 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.Clinical findings sometimes may include positive results for IgM/IgG/IgA Antibodies.

For other causes of lymph node enlargement, see lymphadenopathy.

Diagnosis

It is diagnosed by lymph node excision biopsy.

Kikuchi's disease is a self limiting illness which has symptoms which may overlap with Hodgkin's lymphoma leading to misdiagnosis in some patients.

ANA, APLA, Anti-dsDNA, RF are usually negative, and may help in differentiating from SLE.

Management

No specific cure. Treatment largely supportive. NSAIDs for tender lymphnodes and fever, corticosteroids are useful in severe extranodal or generalized disease.