Kikuchi's Disease of the Mesenteric Lymph Nodes Presenting As Acute
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The Korean Journal of Pathology 2007; 41: 44-6 Kikuchi’s Disease of the Mesenteric Lymph Nodes Presenting as Acute Appendicitis Kyueng-Whan Min∙Ki-Seok Jang Kikuchi’s disease is a benign self-limiting necrotizing lymphadenitis that occurs most commonly Si-Hyong Jang∙Young Soo Song in young women, and is usually found in the cervical lymph nodes. When there is an unusual Woong Na∙Soon-Young Song1 location of involved lymph nodes, the diagnosis can be difficult. We recently treated a patient Seung Sam Paik with Kikuchi’s disease who had ileocecal mesenteric lymph node involvement; the patient pre- sented with symptoms of acute appendicitis in an 11-year old boy. Although mesenteric lymph node involvement of Kikuchi’s disease is very rare, Kikuchi’s disease should be added to the Departments of Pathology and differential diagnosis of acute appendicitis in patients with enlarged ileocecal mesenteric lymph 1Diagnostic Radiology, Hanyang nodes on radiological evaluation. University College of Medicine, Seoul, Korea Received : May 29, 2006 Accepted : August 11, 2006 Corresponding Author Seung Sam Paik, M.D. Department of Pathology, College of Medicine, Hanyang University, 17 Haengdang-dong, Seongdong-gu, Seoul 133-792, Korea Tel: 02-2290-8252 Fax: 02-2296-7502 E-mail: [email protected] Key Words : Kikuchi’s disease; Mesentery; Lymph node; Appendicitis Kikuchi’s disease was first described by Kikuchi1 and Fuji- local clinic. The patient presented with nausea and had vomit- moto et al.2 in 1972. They reported the distinctive histological ed seven times in two days. Fever (39.2℃) and right lower abdo- appearance of the lymph nodes in descriptive terms such as ‘‘lym- minal pain developed one day prior to presentation. The past phadenitis showing focal reticulum cell hyperplasia with nuclear history showed bilateral tonsillectomy due to reactive follicular debris and phagocytosis’’ and ‘‘cervical subacute necrotizing lym- hyperplasia two years ago. Physical examination revealed rebound phadenitis’’. This disorder is usually found in the cervical lymph tenderness in the right lower quadrant of the abdomen. Serum nodes, less frequently in the axillary nodes and rarely presents electrolytes, liver function tests and routine urine analysis were as a generalized lymphadenopathy.3-5 Kikuchi’s disease localized within normal limits. C-reactive protein and erythrocyte sedimen- to the mesenteric lymph nodes is extremely unusual. Only a few tation rate were elevated (2.52 mg/dL, 16 mm/h). The leuko- reports have been previously described in the medical literature.3,6-10 cyte count was elevated at 13,000/ L with 85% of neutrophils. Here we report an additional case of Kikuchi’s disease with ileo- Serologic and microbiologic tests for tuberculosis, systemic lupus cecal mesenteric lymph node involvement that clinically present- erythematosus (SLE), Yersinia infection, cat scratch disease, Tox- ed as acute appendicitis. oplasma lymphadenitis and infectious mononucleosis were not performed. The abdominal plain radiograph was unremarkable. The abdominal computerized tomography showed suspicious CASE REPORT features for acute appendicitis with multiple enlarged mesenteric lymph nodes around the ileocecal area (Fig. 1). With the impres- An 11-year-old boy was transferred to our hospital with the sion of acute appendicitis, the patient underwent laparotomy via impression of acute appendicitis based on the findings of the right oblique skin incision. A normal appearing appendix was 44 Kikuchi’s Disease in Mesenteric Lymph Nodes 45 Fig. 1. An axial scan of contrast-enhanced abdominal CT reveal- Fig. 2. The lymph node showed the characteristic histologic features ed multiple homogeneously enhanced mesenteric lymph nodes of Kikuchi’s disease including marked proliferation of histiocytes, (arrows) at ileocecal area. necrosis with extensive karyorrhexis, and marked phagocytic activity (inset). located at the antececal area. Several enlarged ileocecal lymph nodes were present. The ileocecal mesentery showed mild con- exclude either the clinical diagnosis of tuberculosis or lymphoma.7 gestion. Appendectomy and excision of a mesenteric lymph node The mean age of patients with mesenteric Kikuchi’s disease in were performed. On gross examination, the appendix was unre- previously reported cases was 21.8 years (range, 12-29 years). markable. The mesenteric lymph node was enlarged and mea- Our case was an 11-year-old boy. sured 1 cm in diameter. On microscopic examination, the lymph The histological features of Kikuchi’s disease are characteris- node showed the histological features of Kikuchi’s necrotizing tic. The involved lymph nodes have patchy necrotizing features lymphadenitis including patchy necrotic lesions localized to the localized mainly to the paracortical areas. The necrotizing areas paracortical areas, marked proliferation of benign histiocytes with show eosinophilic fibrinoid material associated with a striking crescent or C-shaped form, necrosis with extensive karyorrhexis, degree of karyorrhexis. Fragments of nuclear debris are distribut- marked phagocytic activity and mild perinodular polymorphous ed irregularly throughout areas of necrosis associated with the infiltrates (Fig. 2). Evaluation with periodic acid-Schiff and sil- presence of apparently atypical mononuclear cells. There is a mix- ver stains, to rule out fungal infection, were negative. The appen- ture in variable proportions of benign histiocytes (so-called cres- dix was well preserved without evidence of acute inflammation. cent or C-shaped forms), immunoblasts, plasmacytoid monocytes, Recovery was uneventful and the patient was discharged three and small lymphocytes. Granulocytes are absent and plasma cells days after surgery. are rare or absent. The capsule is intact and the perinodal adipose tissue may contain a polymorphous infiltrate.4,5,8,11 The histolog- ical findings in our case were consistent with the characteristics DISCUSSION of Kikuchi’s disease. The main differential diagnosis includes several necrotizing Kikuchi’s disease (Necrotizing lymphadenitis) is a well doc- lesions associated with SLE, Yersinia infection, cat scratch dis- umented, benign self-limiting condition that usually presents ease, Toxoplasma lymphadenitis and infectious mononucleosis. with cervical lymphadenopathy and often with a fever of unknown The histological features of SLE include a prominent infiltration origin.1,6,8 The etiology of Kikuchi’s disease remains unknown. of plasma cells, the presence of hematoxyphilic bodies aggregated Although there is no proven etiological agent to date, virus, Tox- toward the edges of the acellular necrotizing areas and the depo- oplasma or Yersinia are currently favored possibilities.5-7 sition of DNA on the vessel walls. In our case, infiltration of The disease occurs much more commonly in young women plasma cells and hematoxyphilic bodies were not present and than men, with most patients being under the age of 40 years.3,5 the blood vessels were unremarkable. A Yersinia infection is char- The classical presentation is one of painful tender cervical lym- acterized by the presence of microabscesses with polymorphonu- phadenopathy associated with pyrexia and frequently arthralgia clear leukocytes localized to germinal centers and aggregates of and rash. The diagnosis is usually made by excision biopsy to epithelioid histiocytes producing a granulomatous reaction. In 46 Kyueng-Whan Min Ki-Seok Jang Si-Hyong Jang, et al. cat-scratch disease, the most characteristic finding is that of stel- remely rare, Kikuchi’s disease with involvement of the ileocecal late microabscesses containing numerous granulocytes surrounded mesenteric lymph nodes should be considered in the differential by pallisading histiocytes. The histological findings of Toxoplas- diagnosis of acute abdominal pain mimicking acute appendicitis. ma lymphadenitis are characterized by florid follicular hyperpla- sia and clusters of epithelioid histiocytes in close proximity or infiltrating reactive germinal centers of follicles, and prominent REFERENCES monocytoid B-cell proliferation within sinuses. Infectious mono- nucleosis shows prominent follicular hyperplasia and a florid 1. Kikuchi M. Lymphadenitis showing focal reticulum cell hyperpla- immunoblastic proliferation in the interfollicular areas.4,11 In our sia with nuclear debris and phagocytes. A clinicopathological study. case, the features of granulomatous inflammation and neutro- Nippon Ketsueki Gakkai Zasshi (Acta Haematol Jpn) 1972; 35: 379- philic infiltration to form an abscess or follicular hyperplasia were 80. not present. 2. Fujimoto Y, Kozima Y, Yamaguchi K. Cervical subacute necrotizing An unusual location of lymph node involvement can make the lymphadenitis. A new clinicopathologic entity. Naika 1972; 30: 920-7. diagnosis of Kikuchi’s disease difficult. Kikuchi’s disease usually 3. Rudniki C, Kessler E, Zarfati M, Turani H, Bar-Ziv Y, Zahavi I. Ki- presents as solitary or multiple enlarged cervical lymph nodes kuchi’s necrotizing lymphadenitis: a cause of fever of unknown ori- and only rarely with generalized lymphadenopathy.3-5,8 Rare sites gin and splenomegaly. Acta Haemat 1988; 79: 99-102. of extracervical lymphadenopathy include axillary, supraclavic- 4. Dorfman RF, Berry GJ. Kikuchi’s histiocytic necrotizing lymphadeni- ular, mediastinal, inguinal, intraparotidal, iliac, celiac, and peri- tis: an analysis of 108 cases with emphasis on differential diagnosis. pancreatic.4 However, localization of Kikuchi’s disease to the Semin