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      Kikuchi-Fujimoto Disease in Michigan: A Rare Case Report and Review of the Literature

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          Abstract

          Background:

          Kikuchi-Fujimoto Disease (KFD), also known as Histiocytic Necrotizing Lymphadenitis, is a rare cause of prolonged cervical lymphadenopathy in both the pediatric and adult populations. It was first reported in Japan in 1972, and since, this disease has been described worldwide, although most cases have been reported in Asia. The etiology of KFD is not fully understood, although there are 2 theories that are described in detail in this review. Kikuchi-Fujimoto Disease typically follows a benign course, with resolution of the lymphadenopathy within 6 months. It is important to recognize KFD as a cause of persistent lymphadenopathy, as it shares many characteristics with and must be differentiated from other causes of lymphadenopathy, including lymphoma, inflammatory disorders, autoimmune conditions, and infectious causes of lymphadenopathy.

          Case presentation:

          Here is presented a case of an 11-year-old male who was born in Vietnam, but subsequently adopted and raised in the United States, who presented to a private practice community-based Otolaryngology group. His chief complaint was a persistent neck mass of approximately 3 months duration. He underwent excisional biopsy for suspected lymphoma, but final pathology rendered a diagnosis of KFD.

          Conclusion:

          The purpose of this article is not only to review the literature but also to contribute awareness of this entity in the differential diagnosis of persistent lymphadenopathy, especially for the general Otolaryngologist in a community-based setting. In addition, this review would be beneficial for other practitioners as well, specifically Pediatricians, Infectious Disease Physicians, Rheumatologists, Pathologists, and Medical Oncologists.

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          Most cited references26

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          Kikuchi-Fujimoto Disease

          Abstract Kikuchi-Fujimoto disease (KFD) is a rare cause of lymphadenopathy, most often cervical. It has been mainly described in Asia. There are few data available on this disease in Europe. We conducted this retrospective, observational, multicenter study to describe KFD in France and to determine the characteristics of severe forms of the disease and forms associated with systemic lupus erythematosus (SLE). We included 91 cases of KFD, diagnosed between January 1989 and January 2011 in 13 French hospital centers (median age, 30 ± 10.4 yr; 77% female). The ethnic origins of the patients were European (33%), Afro-Caribbean (32%), North African (15.4%), and Asian (13%). Eighteen patients had a history of systemic disease, including 11 with SLE. Lymph node involvement was cervical (90%), often in the context of polyadenopathy (52%), and it was associated with hepatomegaly and splenomegaly in 14.8% of cases. Deeper sites of involvement were noted in 18% of cases. Constitutional signs consisted mainly of fever (67%), asthenia (74.4%), and weight loss (51.2%). Other manifestations included skin rash (32.9%), arthromyalgia (34.1%), 2 cases of aseptic meningitis, and 3 cases of hemophagocytic lymphohistiocytosis. Biological signs included lymphocytopenia (63.8%) and increase of acute phase reactants (56.4%). Antinuclear antibodies (ANAs) and anti-DNA antibodies were present in 45.2% and 18% of the patients sampled, respectively. Concomitant viral infection was detected in 8 patients (8.8%). Systemic corticosteroids were prescribed in 32% of cases, hydroxychloroquine in 17.6%, and intravenous immunoglobulin in 3 patients. The disease course was always favorable. Recurrence was observed in 21% of cases. In the 33 patients with ANA at diagnosis, SLE was known in 11 patients, diagnosed concomitantly in 10 cases and in the year following diagnosis in 2 cases; 6 patients did not have SLE, and 4 patients were lost to follow-up (median follow-up, 19 mo; range, 3–39 mo). The presence of weight loss, arthralgia, skin lesions, and ANA was associated with the development of SLE (p < 0.05). Male sex and lymphopenia were associated with severe forms of KFD (p < 0.05). KFD can occur in all populations, irrespective of ethnic origin. Deep forms are common. An association with SLE should be investigated. A prospective study is required to determine the risk factors for the development of SLE.
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            Kikuchi-Fujimoto disease.

            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 disease

              Kikuchi-Fujimoto disease (KFD) is a benign and self-limited disorder, characterized by regional cervical lymphadenopathy with tenderness, usually accompanied with mild fever and night sweats. Less frequent symptoms include weight loss, nausea, vomiting, sore throat. Kikuchi-Fujimoto disease is an extremely rare disease known to have a worldwide distribution with higher prevalence among Japanese and other Asiatic individuals. The clinical, histopathological and immunohistochemical features appear to point to a viral etiology, a hypothesis that still has not been proven. KFD is generally diagnosed on the basis of an excisional biopsy of affected lymph nodes. Its recognition is crucial especially because this disease can be mistaken for systemic lupus erythematosus, malignant lymphoma or even, though rarely, for adenocarcinoma. Clinicians' and pathologists' awareness of this disorder may help prevent misdiagnsois and inappropriate treatment. The diagnosis of KFD merits active consideration in any nodal biopsy showing fragmentation, necrosis and karyorrhexis, especially in young individuals presenting with posterior cervical lymphadenopathy. Treatment is symptomatic (analgesics-antipyretics, non-steroidal anti-inflammatory drugs and, rarely, corticosteroids). Spontaneous recovery occurs in 1 to 4 months. Patients with Kikuchi-Fujimoto disease should be followed-up for several years to survey the possibility of the development of systemic lupus erythematosus.
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                Author and article information

                Journal
                Clin Med Insights Ear Nose Throat
                Clin Med Insights Ear Nose Throat
                ENT
                spent
                Clinical Medicine Insights. Ear, Nose and Throat
                SAGE Publications (Sage UK: London, England )
                1179-5506
                27 February 2019
                2019
                : 12
                : 1179550619828680
                Affiliations
                [1 ]Department of Otolaryngology/Head and Neck Surgery, McLaren Oakland Hospital, Pontiac, MI, USA
                [2 ]North Oakland Ear, Nose & Throat Centers, P.C., Clarkston, MI, USA
                Author notes
                [*]Jeffrey Michael Singh, McLaren Oakland Hospital, 50 North Perry St, Pontiac, IL 48342, USA. Email: Jeffrey.singh@ 123456mclaren.org
                Author information
                https://orcid.org/0000-0001-8283-5113
                Article
                10.1177_1179550619828680
                10.1177/1179550619828680
                6393831
                30833818
                c9ee33eb-dab0-4793-bd5b-51fea7b832c0
                © The Author(s) 2019

                This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 License ( http://www.creativecommons.org/licenses/by-nc/4.0/) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access pages ( https://us.sagepub.com/en-us/nam/open-access-at-sage).

                History
                : 10 December 2018
                : 28 December 2018
                Categories
                Review
                Custom metadata
                January-December 2019

                kikuchi-fujimoto disease,histiocytic necrotizing lymphadenitis,lymphadenopathy,systemic lupus erythematosus,diagnosis,treatment,prognosis

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