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      Kikuchi-Fujimoto Disease in a Young African American Male

      case-report
      1 , 1 , 1 , 1 ,
      ,
      Cureus
      Cureus
      kikuchi-fujimoto disease, histiocytic necrotizing lymphadenitis

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          Abstract

          Kikuchi-Fujimoto disease (KFD), also known as histiocytic necrotizing lymphadenitis, is an extremely rare, self-limiting disorder which typically presents with fever and painful, unilateral cervical lymphadenopathy in previously healthy individuals. Here, we describe a case of KFD which initially presented with fever of unknown origin. Due to its non-specific symptoms and low incidence, KFD poses a diagnostic conundrum for clinicians. Awareness of this disease entity is the key for prompt diagnosis and treatment.

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          Enigmatic Kikuchi-Fujimoto disease: a comprehensive review.

          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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            EBV-associated Kikuchi's histiocytic necrotizing lymphadenitis with cutaneous manifestations.

            The clinical and pathologic findings of Kikuchi's histiocytic necrotizing lymphadenitis may mimic those of malignant lymphoma. We describe a 6-year-old boy with generalized lymphadenopathy, spiking fever, chills, myalgias, malaise, and erythematous, crusted papules. Although cutaneous manifestations have been noted in 16% to 40% of patients with histiocytic necrotizing lymphadenitis, only three publications described skin lesions. The skin lesions and affected lymph nodes revealed histiocytic aggregates, atypical lymphoid cells, karyorrhectic debris, and patchy necrosis. Spontaneous resolution occurred in 2 months. Results of serologic studies, Epstein-Barr virus (EBV) latent membrane protein immunoperoxidase staining, EBER-1 RNA in-situ hybridization, and EBV EBNA-1 DNA polymerase chain reaction implicate EBV as the causative agent.
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              Virus infection in patients with histiocytic necrotizing lymphadenitis in Taiwan. Detection of Epstein-Barr virus, type I human T-cell lymphotropic virus, and parvovirus B19.

              The relationship of Epstein-Barr virus (EBV), type I human T-cell lymphotropic virus (HTLV-I), and parvovirus B19 to histiocytic necrotizing lymphadenitis was studied prospectively in 10 Taiwanese patients using materials obtained by fine-needle aspiration and lymph node biopsy. The presence of EBV was detected by in situ hybridization for EBV-encoded RNA expression. Immunocytochemistry was used to detect virus-encoded protein for EBV and parvovirus B19. DNA in situ hybridization and polymerase chain reaction were performed to determine the existence of HTLV-I provirus. Expressions of EBV-encoded RNA and Fas ligand were detected in all cases. Expression of EBV-encoded protein was identified in only 1 case. Neither HTLV-I nor parvovirus B19 was detected in any case.
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                Author and article information

                Journal
                Cureus
                Cureus
                2168-8184
                Cureus
                Cureus (Palo Alto (CA) )
                2168-8184
                19 April 2018
                April 2018
                : 10
                : 4
                : e2508
                Affiliations
                [1 ] Internal Medicine, University of Connecticut Health Center, Farmington, USA
                Author notes
                Article
                10.7759/cureus.2508
                6007448
                5baad651-763a-4eef-999a-0d54dafdd355
                Copyright © 2018, Zaghloul et al.

                This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

                History
                : 6 April 2018
                : 19 April 2018
                Categories
                Internal Medicine

                kikuchi-fujimoto disease,histiocytic necrotizing lymphadenitis

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