Kikuchi-Fujimoto disease as a rare cause of lymphadenopathy – two cases report and review of current literature

Abstract

Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is a benign and self-limited disease, characterized typically by enlargement of regional lymph nodes accompanied by fever. KFD affects predominantly young adult females of Asian origin and is rarely seen in European countries, where it may cause diagnostic difficulties. Two cases of KFD in a 33 and 27-year-old woman with mild fever, malaise, lymphadenopathy initially misdiagnosed for indolent non-Hodgkin's lymphoma was presented. The definitive diagnosis was established on the basis of histopathological examination of totally excised cervical lymph nodes. The propriety diagnosis allowed us to avoid inappropriate chemotherapy. The disease course in our patient was uneventful during the 1.5 and 12-year follow-up period. The clinical presentations, complications as well as current concepts on pathogenesis, diagnosis and treatment of the Kikuchi-Fujimoto disease was briefly reviewed in this paper. The need of a long-term follow-up of patients with Kikuchi-Fujimoto disease was emphasized.

Quiz. Correct answer to the quiz. Check your diagnosis Histiocytic necrotising lymphadenitis in mediastinum mimicking thymoma or lymphoma – case presentation and literature review of Kikuchi Fujimoto disease

“…The surroundings of these foci are filled with the full number of characteristic CD68-positive plasmacytoid histiocytes containing comma-shaped nuclei and transformed lymphocytes (immunoblasts). Neutrophils, eosinophils or plasma cells are complitely absent [3,10,13].…”

“…In view there are frequent simultaneous occurrences of autoimmune diseases with histiocytic necrotizing lymphadenitis. The most common are systemic lupus erythematosus, Hashimoto's disease and rheumatoid arthritis [3,19].…”

“…The increased disease incidence of Asian race has been associated with a group of HLA class II genes, such as DPA1 * 01, and DPB1 * 0202, predisposing to the development of Kikuchi Fujimoto disease [3,13,23].…”

“…In Europe, it is extremely rare. In Poland only 11 cases were described in literature [3,4,5,6,7,8]. The etiology is not well understood but genetics, autoimmune and inflammatory factors are taken into account.…”

State of the globe: Time to revisit kikuchi Fujimoto disease

“…[ 1 ] A small percentage of patients may develop flu-like symptoms, skin rash, hepatitis, arthritis, and oral ulcers. [ 2 3 ]…”