Kikuchi-Fujimoto Disease in the United States: Three Case Reports and Review of the Literature

Abstract

Kikuchi-Fujimoto Disease (KFD), also known as histiocytic lymphadenitis, is a benign, self-limiting disease that manifests primarily as cervical lymphadenopathy but may include low-grade fever, headache, and fatigue. There is a higher incidence of KFD in women aged 20–35 years and in Asian populations. A PubMed search revealed 590 articles that described KFD. Of these, 22 cases have been fully described in the United States. Ten of the 22 (45%) patients were male and 12 (55%) were female, with 20% Caucasian, 20% Asian American, and the remaining 60% of other ethnic backgrounds. In this study, we describe an additional 3 cases of KFD and discuss the diagnosis, pathology, and management of KFD.

Kikuchi-Fujimoto Disease: Unusual Presentation of Rare Disease

“…2 Less common symptoms include chills, night sweats, weight loss, arthralgia, and cutaneous manifestations. 2,5 Although the amount of weight loss is not mentioned in the literature, our patient had lost a significant amount of weight. This may be explained by lack of appetite due to an inflammatory response and release of cytokines.…”

“…Differential diagnosis includes lymphoproliferative disorders, systemic lupus erythematosus (SLE), and infection. 5 Excision biopsy of affected lymph nodes is essential for diagnosis. Histopathological findings include paracortical expansion with foci of coagulative necrosis and extensive karyorrhectic debris.…”

Kikuchi Fujimoto Disease: The First Reported Case in Mongolia

“…Atypical lymphocytes may also be present in the peripheral blood [4,14]. The diagnosis of KFD is usually based on lymph node histology, which shows a patchy, necrotizing process affecting the paracortical areas of the lymph node, with various types of histiocytes and plasmacytoid monocytes, particularly foamy histiocytes, are predominantly present around foci of necrosis [4,7].…”

“…Typically, the patient has associated fever, night sweats, and weight loss [14]. Extranodal involvement of this disease, including skin, eye and bone marrow localisations, has rarely been described.…”

“…Facial or malar "butterfly rash" may also be present. Skin involvement tends to occur in patients who have more severe and protracted course [14,15].…”