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      Kikuchi–Fujimoto disease and systemic lupus erythematosus

      case-report

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          Abstract

          Kikuchi–Fujimoto disease, or histiocytic necrotizing lymphadenitis, is an infrequent idiopathic disorder. It has been associated with autoimmune disorders, of which systemic lupus erythematosus is the most outstanding. The basis of its diagnosis relies on the histological examination of lymph nodes, which typically reveals necrosis surrounded by histiocytes with crescentic nucleus, immunoblasts and plasma cells, and absence of neutrophils. We report the case of a 27-year-old Argentinian female patient without any relevant past medical history to demonstrate the correlation between Kikuchi–Fujimoto disease and systemic lupus erythematosus.

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          Kikuchi-Fujimoto disease.

          Edward Hutchinson, Endi Wang (2010)
          Kikuchi-Fujimoto disease, or histiocytic necrotizing lymphadenitis, is a self-limited condition, characterized by benign lymphadenopathy with associated fevers and systemic symptoms. It most commonly affects adults younger than 40 years of age and of Asian descent. Involved lymph nodes demonstrate paracortical areas of apoptotic necrosis with abundant karyorrhectic debris and a proliferation of histiocytes, plasmacytoid dendritic cells, and CD8(+) T cells in the absence of neutrophils. Kikuchi-Fujimoto disease is thought to have 3 evolving phases: proliferative, necrotizing, and xanthomatous. The etiology is unknown, although viruses and autoimmune mechanisms have been proposed. No specific laboratory tests contribute to the diagnosis. Diagnosis requires histopathologic examination and exclusion of other factors by ancillary studies. Non-Hodgkin lymphoma and systemic lupus erythematosus should be ruled out before diagnosis of Kikuchi-Fujimoto disease, given the overlapped clinical and histologic features as well as the different therapeutic approaches. Treatment involves supportive measures, and the symptoms usually resolve spontaneously within 4 months.
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            Kikuchi-Fujimoto's disease associated with systemic lupus erythematosus: case report and review of the literature.

            Mittermayer Santiago, Bruno Lessa, A. L. B. D. Santana … (2005)
            Kikuchi-Fujimoto's disease (KFD) or histiocytic necrotising lymphadenitis is a benign and self-limited disease, of unknown aetiology, which affects mainly young women. It presents with localised lymphadenopathy, predominantly in the cervical region, accompanied by fever and leukopenia in up to 50% of the cases. KFD has been rarely described in association with systemic lupus erythematosus (SLE), and its diagnosis can precede, postdate or coincide with the diagnosis of SLE. We present a patient with the diagnosis of SLE characterised by arthritis, leukopenia, malar rash, photosensitivity and positive ANA, besides cervical lymphadenopathy whose biopsy was compatible with KFD, which improved after using prednisone. Although the presence of lymphadenopathy is not uncommon in SLE patients, particularly in the phases of disease activity, the concomitance with KFD has rarely been reported in the literature. Its recognition is necessary because one can avoid laborious investigation for infectious and lymphoproliferative diseases.
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              Fatal Kikuchi-Fujimoto disease associated with SLE and hemophagocytic syndrome: a case report.

              Thatchai Kampitak (2008)
              Kikuchi-Fujimoto disease (KFD) or histiocytic necrotizing lymphadenitis is a rare, benign, autoimmune condition characterized by lymphadenopathy, fever, and neutropenia. KFD can frequently mimic various diseases including infection, malignancy, and autoimmune disease. KFD has also been infrequently reported in association with SLE, which can be diagnosed previously, simultaneously, or after the diagnosis of SLE. The author presents here the first case of fatal KFD simultaneously occurred with SLE complicated with hemophagocytic syndrome and severe infection. The recognition of this association is of significance for management of affected patients with early intensive immunosuppressive therapy for favorable outcome.
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                Author and article information

                Journal
                Journal ID (nlm-ta): Int Med Case Rep J
                Journal ID (iso-abbrev): Int Med Case Rep J
                Journal ID (publisher-id): International Medical Case Reports Journal
                Title: International Medical Case Reports Journal
                Publisher: Dove Medical Press
                ISSN (Electronic): 1179-142X
                Publication date Collection: 2016
                Publication date (Electronic): 29 June 2016
                Volume: 9
                Pages: 163-167
                Affiliations
                [1 ]Clinical Medicine Department
                [2 ]Rheumatology Department
                [3 ]Pathology Department, Hospital Privado Universitario de Córdoba Medical Center
                [4 ]Instituto Universitario de Ciencias Biomédicas, Universitary Institute, Córdoba, Argentina
                Author notes
                Correspondence: Diego F Baenas, Clinical Medicine Department, Hospital Privado Universitario de Córdoba, Naciones Unidas 446 Street, Córdoba 5016, Argentina, Tel +54 351 468 8200, Email baenashospitalprivado@ 123456gmail.com
                Article
                Publisher ID: imcrj-9-163
                DOI: 10.2147/IMCRJ.S106396
                PMC ID: 4935008
                PubMed ID: 27418858
                SO-VID: ceb13961-5490-441f-95eb-fd0fc003ca04
                Copyright © © 2016 Baenas et al. This work is published and licensed by Dove Medical Press Limited
                License:

                The full terms of this license are available at https://www.dovepress.com/terms.php and incorporate the Creative Commons Attribution – Non Commercial (unported, v3.0) License ( http://creativecommons.org/licenses/by-nc/3.0/). By accessing the work you hereby accept the Terms. Non-commercial uses of the work are permitted without any further permission from Dove Medical Press Limited, provided the work is properly attributed.

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                Categories
                Subject: Case-Report

                Keywords: histiocytic necrotizing lymphadenitis,systemic lupus erythematosus,autoimmune disorders,febrile syndrome
                Data availability:
                Keywords: histiocytic necrotizing lymphadenitis, systemic lupus erythematosus, autoimmune disorders, febrile syndrome

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