Kikuchi-Fujimoto Disease

Case: 24 years old female is admitted to ICU with fever, cervical lymphadenopathy and sepsis. Subsequent workup led to the diagnosis of Kikuchi disease.

Answer: Kikuchi disease is also known as Kikuchi-Fujimoto disease. It's proper nomenclature is  Kikuchi histiocytic necrotizing lymphadenitis due to its histopathological finding. 

Usually patients get admitted with unknown cause of fever and cervical lymphadenopathy. In most cases, biopsy of the involved node leads to the diagnosis. It is usually a benign disease. Contrary to popular belief it is found worldwide. Major symptoms are lymphadenopathy, leukopenia, fever, rash, arthritis, fatigue, and rarely hepatosplenomegaly. It may also present as aseptic meningitis.

Although various treatments have been tried such as high-dose glucocorticoids, hydroxychloroquine, and interleukin 1 inhibitor, there is no established effective treatment.

Its clinical significance lies in the fact that these patients are more prone to develop systemic lupus erythematosus (SLE), and recurrences of Kikuchi disease.

#lymphadenopathies

References:

1. Perry AM, Choi SM. Kikuchi-Fujimoto Disease: A Review. Arch Pathol Lab Med. 2018 Nov;142(11):1341-1346. doi: 10.5858/arpa.2018-0219-RA. PMID: 30407860.

2. Alam H, Saeed MO, Saeed AB, Zaidi A. Kikuchi disease. J Pak Med Assoc. 2015 Dec;65(12):1349-50. PMID: 26627522.