Kikuchi disease

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Histiocytic necrotizing lymphadenitis - intermed mag

Kikuchi disease

Kikuchi disease, also known as Kikuchi-Fujimoto disease or histiocytic necrotizing lymphadenitis, is a rare, benign condition characterized by lymphadenopathy, fever, and night sweats. It was first described in Japan by Dr. Masahiro Kikuchi and independently by Dr. Y. Fujimoto in 1972.

Presentation

Kikuchi disease primarily affects young adults, particularly women under the age of 30. The most common symptom is lymphadenopathy, typically involving the cervical lymph nodes. Other symptoms may include fever, night sweats, weight loss, and fatigue. Some patients may also experience rash and joint pain.

Pathophysiology

The exact cause of Kikuchi disease is unknown, but it is believed to be related to an abnormal immune response. Histologically, the affected lymph nodes show areas of necrosis with abundant histiocytes and a paucity of neutrophils. The disease is self-limiting and usually resolves within a few months.

Diagnosis

Diagnosis of Kikuchi disease is primarily based on histopathological examination of an excised lymph node. The characteristic findings include necrotizing lymphadenitis with karyorrhectic debris and an absence of neutrophils. Differential diagnosis includes systemic lupus erythematosus, lymphoma, and infectious causes of lymphadenopathy.

Treatment

There is no specific treatment for Kikuchi disease. Management is generally supportive, including the use of nonsteroidal anti-inflammatory drugs (NSAIDs) for pain and fever. In severe cases, corticosteroids may be prescribed. The prognosis is excellent, with most patients recovering fully without long-term complications.

Epidemiology

Kikuchi disease is more common in Asia, particularly in Japan, but cases have been reported worldwide. It predominantly affects young adults, with a higher incidence in females.

Related Pages

References

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