Kikuchi-Fujimoto Disease: A Review

Abstract

Kikuchi-Fujimoto disease (KFD) is a rare entity characterized by subacute necrotizing lymphadenopathy and frequently associated with fever. Young adults of Asian ancestry are most commonly affected, but it has been reported worldwide. Despite many studies in the literature, the cause of KFD remains uncertain. Histologically, KFD is characterized by paracortical lymph node expansion with patchy, well-circumscribed areas of necrosis showing abundant karyorrhectic nuclear debris and absence of neutrophils and eosinophils. Three evolving histologic patterns—proliferative, necrotizing, and xanthomatous—have been recognized. By immunohistochemistry, histiocytes in KFD are positive for myeloperoxidase. There is a marked predominance of T cells in the lesions (with mostly CD8-positive cells) with very few B cells. The differential diagnosis of KFD includes infectious lymphadenitis, autoimmune lymphadenopathy (primarily systemic lupus erythematosus), and lymphoma. Clinicians and pathologists are poorly familiar with this entity, which frequently causes significant diagnostic challenges.

Case Report of Kikuchi-Fujimoto Disease from Sub-Saharan Africa: An Important Mimic of Tuberculous Lymphadenitis

“…Kikuchi-Fujimoto disease, also known as histiocytic necrotizing lymphadenitis, was first described in 1972 [1]. It is a rare self-limiting lymphadenopathy, more commonly described in females, those aged 20-40 years and are of Southeast Asia origin [2,3]. Although less common within the African population, a few cases of the disease have been reported from Gabon, Senegal, Morocco, and Tunisia [4][5][6].…”

“…Xu et al identified CMV,EBV, HHV-6, HHV-7, Parvovirus B19, and mycobacterial species from lymph node samples of 153 patients in China with KFD [9]. KFD has been described in association with SLE, Still's disease, Wegener's granulomatosis, Graves' disease, and Sjogren's disease [3]. Tanaka et al found higher frequencies of the HLA DPA1 * 01 and DPB1 * 0202 allele in Japanese patients with KFD [10].…”

“…e histiocytes in KFD are positive for lysozyme, myeloperoxidase, CD68, CD163, and CD4. e lymphocytes in the lesions are mostly CD3-positive T cells demonstrating a predominance of CD8 compared with CD4, with very few CD20-positive B cells [3]. In contrast, lymphadenitis from TB classically has the presence of well/poorly defined granulomas with areas of caseous necrosis [27].…”

A Case of Kikuchi-Fujimoto Disease Associated with Erosive Lichen Planus

“…The most common region of lymph node involvement is posterior cervical (60% -90% of cases) and frequently occurs with concomitant involvement of axillary and/or supraclavicular lymph nodes. Generalized lymphadenopathy, however, is rarely reported [2,5,[8][9]. Intra-abdominal regions have also been reported with unknown frequency.…”

“…Lymphadenopathy is most frequently associated with fever and other less commonly reported signs and symptoms, including weight loss, vomiting, headache, arthralgia, night sweats, sore throat, hepatosplenomegaly, and cutaneous lesions [2,[8][9]. Cutaneous eruptions in KFD occur in about 40% of cases with reported manifestations, including urticarial-like lesions, generalized erythema and papules, plaques and nodules, leukocytoclastic vasculitis, erythema multiforme, indurated erythematous lesions, oral ulcerations, alopecia, and malar erythema [2,5,9,[11][12]. Furthermore, the cutaneous manifestations of KFD could be consistent with cutaneous manifestations of SLE [13].…”