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      Fatal Kikuchi-like lymphadenitis associated with connective tissue disease: a report of two cases and review of the literature

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          Abstract

          Introduction

          Kikuchi-Fujimoto disease, is usually a benign self-limiting disease which typically affects young females under the age of 30 years and resolves without treatment within six months. However, when it occurs in the context of connective tissue disease, particularly systemic lupus erythematosus (SLE), it is usually associated with a flare-up of the patient’s symptoms, requiring treatment, and can lead to severe, potentially life-threatening sequelae.

          Case description

          Here, we report and compare two cases of unclassifiable connective tissue disease who developed a Kikuchi-like lymphadenitis and sepsis-like clinical syndrome, including disseminated intravascular coagulation, which proved rapidly fatal.

          Discussion and evaluation

          In our review of the literature, we found 55 cases of Kikuchi-Fujimoto disease occurring in the context of definite connective tissue disease, 50 of which were associated with SLE. Of the 55 cases, 22 (40%) had simultaneous onset with, 19 (35%) predated the onset of and 14 (25%) developed after the associated connective tissue disease. Life-threatening autoimmune sequelae were reported in 8 cases, 2 of which were fatal. The aetiology of the association remains unknown.

          Conclusion

          Kikuchi-Fujimoto disease is a histopathological diagnosis, and although the classical form appears to represent a distinct entity, it is unclear whether it is always the same entity, regardless of the context in which it occurs, or whether it represents a histological pattern with a variety of possible causes. In any case, the possibility of auto-immune sequelae in patients with known autoimmune disease should always be considered if these patients present with a sepsis-like clinical syndrome and no infective source is identified.

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          Most cited references 51

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          Kikuchi-Fujimoto Disease: analysis of 244 cases.

          Kikuchi-Fujimoto Disease (KFD) was first described in Japan in 1972. The disease frequently mimics tuberculous lymphadenitis, malign lymphoma, and many other benign and malignant conditions. To our knowledge, there is no previous study comparing the clinical and laboratory characteristics of patients from different geographical parts of the world. We searched literature records beginning from 1991 and analyzed epidemiological, clinical, and laboratory data of 244 patients (including cases diagnosed in our institution) reported in 181 publications. Of the 244 cases, 33% were male and 77% were female. Mean age was 25 (1-64) and 70% was younger than 30. Most of the cases were reported from Taiwan (36%), USA (6.6%), and Spain (6.3%). Fever (35%), fatigue (7%) and joint pain (7%) were the most frequent symptoms, while lymphadenomegaly (100%), erythematous rashes (10%), arthritis (5%), hepatosplenomegaly (3%), leucopenia (43%), high erythrocyte sedimentation rate (40%), and anemia (23%) being the most common findings. KFD was associated with SLE (32 cases), non-infectious inflammatory diseases (24 cases), and viral infections (17 cases). SLE was more frequent in cases from Asia than Europe (28 and 9%, respectively). The disease was self-limiting in 156 (64%) and corticosteroid treatment was necessary in 16 (16%) of the cases. The mortality rate was 2.1%. Early diagnosis is crucial as the clinical and laboratory presentation generally imitates situations needing lengthy and costly diagnostic and therapeutic interventions. Additionally, association with SLE needs further investigation.
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            Subacute cutaneous lupus erythematosus: a cutaneous marker for a distinct lupus erythematosus subset.

            We have characterized the clinical and laboratory features of 27 patients who had in common a recurring, superficial, nonscarring type of cutaneous lupus erythematosus (LE) that occurred in a characteristic distribution (subacute cutaneous lupus erythematosus [SCLE]). This clinically distinct form of cutaneous LE has not previously been analyzed as a separate entity and thus, its clinical importance has not been fully appreciated. We found that these patients frequently had a mild systemic illness marked by musculoskeletal complaints and serologic abnormalities. Forty-eight percent had systemic LE by American Rheumatism Association criteria; however, none had serious CNS or renal disease. Thus, those with SCLE are a subset of patients with LE who generally have an illness intermediate in severity between discoid LE and severe systemic LE.
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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

                Contributors
                Vijay.Sharma1@nhs.net
                rosslyn.rankin@nhs.net
                Journal
                Springerplus
                Springerplus
                SpringerPlus
                Springer International Publishing (Cham )
                2193-1801
                8 April 2015
                8 April 2015
                2015
                : 4
                Affiliations
                [ ]Department of Pathology, Raigmore Hospital, Inverness, Scotland
                [ ]Department of Pathology, Aberdeen Royal Infirmary, Foresterhill Aberdeen, Scotland
                [ ]Division of Applied Medicine, School of Medicine and Dentistry, University of Aberdeen, Aberdeen, Scotland
                Article
                925
                10.1186/s40064-015-0925-7
                4398681
                © Sharma and Rankin; licensee Springer. 2015

                This is an Open Access article distributed under the terms of the Creative Commons Attribution License ( http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited.

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                © The Author(s) 2015

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