Kikuchi disease

Infobox_Disease
Name = Kikuchi disease


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

It was first described in Japan by Dr M. Kikuchi in 1972 [Kikuchi M. Lymphadenitis showing focal reticulum cell hyperplasia with nuclear debris and phagocytes. "Acta Hematol Jpn" 1972;35:379–80. ] and independently by Fujimoto.

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 slight female preponderance. 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 viral candidates have been proposed to be associated with Kikuchi's disease, including cytomegalovirus, Epstein Barr virus, Herpes simplex virus, Varicella zoster virus, parainfluenza virus, parvovirus B19 and paramyxovirus. Association between 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.

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 DS DNA, 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.

References


Wikimedia Foundation. 2010.

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  • Histiocytic necrotizing lymphadenitis — A disorder, also called Kikuchi disease, that typically causes swollen glands in the neck (cervical lymphadeniopathy) together with fever or flu like symptoms. Laboratory test abnormalities include elevated erythrocyte sedimentation rate (ESR),… …   Medical dictionary


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