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      Take a second look: it’s Kikuchi’s disease! A case report and review of literature

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          Abstract

          Generalized lymphadenopathy is a common cause of concern for both patients and clinicians. Possible etiologies include infections, malignancies and autoimmune diseases. Kikuchi Fujimoto disease (KFD) is a hyperergic condition that presents with fever, lymphadenopathy and can include systemic involvement, thus being easily mistaken for the above-mentioned entities. We report the case of a previously healthy 18- year old male who presented with a selflimiting generalized lymphadenopathy, high fevers, skin vasculitis and polyserositis. The lymph-node biopsy revealed a histiocytotic necrotizing lymphadenitis, suggestive of Kikuchi’s disease. This case emphasizes the importance of KFD in the differential diagnosis of lymphadenopathy, especially in young adults.

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          Enigmatic Kikuchi-Fujimoto disease: a comprehensive review.

          Xavier F Bosch, Elias Campo, Rosa Miquel … (2004)
          To determine the clinicopathologic significance of Kikuchi-Fujimoto disease (KFD) and review the literature on this condition, we conducted a MEDLINE search of English-language articles published between 1972 and December 2003. KFD has a worldwide distribution, and Asiatic people have a higher prevalence. Its pathogenesis remains controversial. Patients are young and seek care because of acute tender, cervical lymphadenopathy and low-grade fever. Histologic findings include paracortical areas of coagulative necrosis with abundant karyorrhectic debris. Karyorrhectic foci consist of various types of histiocytes, plasmacytoid monocytes, immunoblasts, and small and large lymphocytes. There is an abundance of T cells with predominance of CD8+ over CD4+ T cells. Differential diagnosis includes lymphoma, lymphadenitis associated with systemic lupus erythematosus, and even adenocarcinoma. KFD is an uncommon, self-limited, and perhaps underdiagnosed process with an excellent prognosis. Accurate clinicopathologic recognition is crucial, particularly because KFD can be mistaken for malignant lymphoma.
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            Kikuchi's histiocytic necrotizing lymphadenitis: an analysis of 108 cases with emphasis on differential diagnosis.

            Jay G. Berry, R F Dorfman (1988)
            Kikuchi's necrotizing lymphadenitis has now become recognized in many parts of the world as a well-defined clinicopathologic entity with a remarkable predilection for cervical lymph nodes of young women. The morphologic features encountered in lymph nodes are distinctive and should enable pathologists to establish the diagnosis with confidence. Nonetheless, this analysis of 108 cases, encountered over a 15-year period, has emphasized the difficulty that many pathologists have in recognizing this disorder, and in particular, in distinguishing it from malignant lymphoma. Although in the vast majority of cases, lymphadenopathy and other symptomatology resolves spontaneously, two of our patients, thought initially to have Kikuchi's disease, developed systemic lupus erythematosus. This raises consideration for the proposal that Kikuchi's disease may reflect a self-limited SLE-like auto-immune condition (a "forme fruste" of SLE), perhaps induced by virus-infected transformed lymphocytes. Moreover, such observations indicate that patients with Kikuchi's disease should be kept under observation for several years to ensure that they are not at risk for the development of systemic lupus erythematosus.
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              Autoimmunity and primary immunodeficiency: two sides of the same coin?

              Reinhold Schmidt, Bodo Grimbacher, Torsten Witte (2017)
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                Author and article information

                Journal
                Journal ID (nlm-ta): Clin Pract
                Journal ID (publisher-id): CP
                Title: Clinics and Practice
                Publisher: PAGEPress Publications, Pavia, Italy
                ISSN (Print): 2039-7275
                ISSN (Electronic): 2039-7283
                Publication date (Electronic): 12 December 2018
                Publication date Collection: 26 October 2018
                Volume: 8
                Issue: 4
                Electronic Location Identifier: 1095
                Affiliations
                [1 ]Department of Immunology and Rheumatology
                [2 ]Department of Pathology, Hannover Medical University , Hannover, Germany
                Author notes
                Department of Immunology and Rheumatology, Hannover Medical University, Carl-Neuberg-Str. 1, 30625 Hannover, Germany. +49.0.511.532.6656+49.0.511.532.9067. Joean.Oana@ 123456mh-hannover.de

                Contributions: OJ treated the patient, designed and wrote the manuscript, prepared the figures; TT treated the patient, designed and wrote the manuscript; MR performed and interpreted the histological stainings; RES and MS supervised OJ and TT, discussed the differential diag-nosis, provided critical input for the manuscript. All authors read and approved the contents of the manuscript.

                Conflict of interest: the authors declare no potential conflict of interest.

                Article
                DOI: 10.4081/cp.2018.1095
                PMC ID: 6297864
                PubMed ID: 30631410
                SO-VID: 44bc5b63-ee18-4b8f-9817-c301c65fbdfa
                Copyright © ©Copyright O. Joean et al., 2018
                License:

                This work is licensed under a Creative Commons Attribution NonCommercial 4.0 License (CC BY-NC 4.0).

                History
                Date received : 08 July 2018
                Date accepted : 05 December 2018
                Page count
                Figures: 2, Tables: 0, Equations: 0, References: 20, Pages: 3
                Funding
                Funding: funding was provided by the Hannover Medical University, Hannover, Germany.
                Categories
                Subject: Case Report

                Keywords: kikuchi’s disease,necrotising histiocytic lymphadenitis
                Data availability:
                Keywords: kikuchi’s disease, necrotising histiocytic lymphadenitis

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