Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Histiocytic necrotizing lymphadenitis or Kikuchi-Fujimoto disease is a rare entity, and even more rarely, it is associated with other diseases. In a few cases, the condition is associated with cutaneous lupus erythematosus (CLE). Histiocytic necrotizing lymphadenitis predominantly affects young women, who present with solitary or multiple cervical lymphadenopathy accompanied by symptoms such as fever, weight loss, sweating, or, in exceptional cases, hepatosplenomegaly. Laboratory examinations show normal or nonspecific results. The disease is of unknown origin, although a viral origin has been suggested, with the suspected agents including Epstein-Barr virus, herpesvirus type 6, and cytomegalovirus (CMV). Although the first and most of the more recent cases have been reported in Oriental patients, the disease has a wide geographic distribution. The clinical evolution is favorable, with spontaneous remission in less than 4 months in almost all cases. We present a case of a 37-year-old woman from Peru who presented with cervical adenopathies on two occasions. Biopsy of a lymph node revealed a histopathologic picture compatible with Kikuchi-Fujimoto histiocytic necrotizing lymphadenitis. The adenopathy disappeared in a few months. A year later, she presented with a maculopapular rash in the nasal and malar regions. The results of the skin biopsy and immunofluorescence examination were compatible with chronic CLE. The results of the serology testing for CMV were positive. Treatment with chloroquine was initiated, with almost complete recovery by 5 months. No manifestations of systemic lupus erythematosus have occurred since. The epidemiologic, clinical, and anatomopathologic aspects as well as the differential diagnosis of this entity are reviewed.
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PMID:Kikuchi-Fujimoto necrotizing lymphadenitis associated with cutaneous lupus erythematosus: a case report. 1094 58

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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PMID:Kikuchi-Fujimoto Disease: analysis of 244 cases. 1653 88