Copyright ©The Author(s) 2023. Published by Baishideng Publishing Group Inc. All rights reserved.
World J Clin Cases. Jun 6, 2023; 11(16): 3664-3679
Published online Jun 6, 2023. doi: 10.12998/wjcc.v11.i16.3664
Kikuchi-Fujimoto disease: A comprehensive review
Vikram K Mahajan, Vikas Sharma, Neeraj Sharma, Ritu Rani
Vikram K Mahajan, Vikas Sharma, Neeraj Sharma, Ritu Rani, Department of Dermatology, Venereology and Leprosy, Dr. Radhakrishnan Government Medical College, Hamirpur 177001, Himachal Pradesh, India
Author contributions: Mahajan VK planned, obtained, compiled, analyzed and interpreted the data, prepared the draft, and critically evaluated the manuscript for important intellectual content; Sharma V, Sharma N, and Rani R helped in planning, compiling, and preparing the draft; all authors were involved in the revision of the manuscript and agreed to the final content.
Conflict-of-interest statement: All the authors report no relevant conflicts of interest for this article.
Open-Access: This article is an open-access article that was selected by an in-house editor and fully peer-reviewed by external reviewers. It is distributed in accordance with the Creative Commons Attribution NonCommercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See:
Corresponding author: Vikram K Mahajan, MD, Professor, Department of Dermatology, Venereology and Leprosy, Dr. Radhakrishnan Government Medical College, Hamirpur 177001, Himachal Pradesh, India.
Received: December 27, 2022
Peer-review started: December 27, 2022
First decision: March 24, 2023
Revised: March 29, 2023
Accepted: April 27, 2023
Article in press: April 27, 2023
Published online: June 6, 2023

Kikuchi-Fujimoto disease, a rare form of necrotizing lymphadenitis, is an uncommon, benign, self-limiting disorder of obscure etiology. It affects mostly young adults of both genders. Clinically, it presents with fever and lymphadenopathy of a firm to rubbery consistency frequently involving cervical lymph nodes while weight loss, splenomegaly, leucopenia, and elevated erythrocyte sedimentation rate feature in severely affected patients. Cutaneous involvement occurs in about 30%-40% of cases as facial erythema and nonspecific erythematous papules, plaques, acneiform or morbilliform lesions of great histologic heterogeneity. Both Kikuchi-Fujimoto disease and systemic lupus erythematosus share an obscure and complex relationship as systemic lupus erythematosus may occasionally precede, develop subsequently, or sometimes be associated concurrently with Kikuchi-Fujimoto disease. It is often mistaken for non-Hodgkin lymphoma while lupus lymphadenitis, cat-scratch disease, Sweet’s syndrome, Still’s disease, drug eruptions, infectious mononucleosis, and viral or tubercular lymphadenitis are other common differentials. Fine needle aspiration cytology mostly has features of nonspecific reactive lymphadenitis and immunohistochemistry studies usually show variable features of uncertain diagnostic value. Since its diagnosis is exclusively from histopathology, it needs to be evaluated more carefully; an early lymph node biopsy will obviate the need for unnecessary investigations and therapeutic trials. Its treatment with systemic corticosteroids, hydroxychloroquine, or antimicrobial agents mostly remains empirical. The article reviews clinicoepidemiological, diagnostic, and management aspects of KFD from the perspective of practicing clinicians.

Keywords: Cervical lymphadenopathy, Histiocytic necrotizing lymphadenitis, Kikuchi’s disease, Lymphadenopathy, Lymphoma, Systemic lupus erythematosus

Core Tip: Kikuchi-Fujimoto disease or Kikuchi disease described originally in young Japanese women is a rare benign cause of fever and lymphadenopathy usually involving cervical lymph nodes. The disease has been reported worldwide in both genders across ethnic and age groups. Histopathologically, histiocytic necrotizing lymphadenitis is characteristic and needs differentiation from more serious conditions such as malignant lymphoma in acute or subacute form. Its long-term prognosis is favorable albeit long-term follow-up is recommended because of a rare but increased risk of developing systemic lupus erythematosus/other autoimmune disorders. Patients with a severe or recurrent disease need treatment with nonsteroidal anti-inflammatory drugs, systemic corticosteroids, and/or other immunomodulators.