Kikuchi Disease Workup

  • Author: John Boone, MD; Chief Editor: Emmanuel C Besa, MD   more...
 
Updated: Jan 10, 2012
 

Laboratory Studies

  • In patients with Kikuchi disease, diagnostic laboratory and radiologic test findings are nonspecific. Although results of fine-needle aspiration (FNA) may be suggestive,[23, 24] the diagnosis of Kikuchi disease is confirmed only by excisional lymph node biopsy.
    • Complete blood cell (CBC) count
      • Mild granulocytopenia is observed in 20-50% of patients. Leukocytosis is present in 2-5% of patients.
      • Atypical lymphocytes are observed in 25% of patients.
    • Erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) levels may be elevated.
    • Elevated LDH levels suggest hepatic involvement.
    • Results from autoimmune antibody studies, including LE preparation and RF and ANA studies, are generally negative. These findings may help the clinician distinguish Kikuchi disease from SLE.
Next

Imaging Studies

  • Diagnostic imaging studies confirm the presence of enlarged lymph nodes in the affected areas, but they cannot specifically confirm a diagnosis of Kikuchi disease.
    • Computed tomography (CT) scanning and magnetic resonance imaging (MRI)
      • Uniform enlargement of lymph nodes in affected areas is noted.
      • Postcontrast enhancement may be observed.
      • Kwon et al reported CT findings in 96 people with Kikuchi disease.[25] The authors found homogeneous lymph node enlargement in 83.3% of the patients, perinodal infiltration in 81.3%, and prominent areas of low attenuation suggestive of focal necrosis in 16.7%.
      • Han et al examined the clinical characteristics and CT scan features of 15 pediatric patients (age 2-14 y) with Kikuchi disease.[26] The investigators noted a 10-day median duration of fever; all patients had cervical lymphadenopathy, of which 14 (93%) children demonstrated perinodal infiltrates and 10 (63%) patients demonstrated nonenhancing necrosis. Fourteen children had improvement of their condition, of which 4 patients had recurrent episodes of Kikuchi disease.[26]
    • Ultrasonography[27]
      • Enlarged nodes may be either homogeneous or heterogeneous.
      • The nodes often have hyperechoic rims.
    • Chest radiography: Although radiographic findings are generally unremarkable in Kikuchi disease, a chest radiograph is recommended in the evaluation of cervical adenopathy to look for evidence of tuberculosis or malignancy.[8]
Previous
Next

Procedures

  • A definitive diagnosis of Kikuchi disease can be made only by tissue evaluation. Cytologic examination by FNA can suggest the diagnosis of Kikuchi disease, especially when supported by typical clinical findings, but excisional biopsy of an involved lymph node is needed to confirm the diagnosis in doubtful cases.
  • FNA
    • FNA findings are most often nonspecific, Although some authors believe that the diagnosis can be confirmed when supported by typical clinical findings. Most authors recommend confirmation by excisional biopsy.
    • In a retrospective study of 44 patients, FNA had an overall accuracy of 56.75% in diagnosing Kikuchi disease.[23]
    • Characteristic cytologic findings in Kikuchi disease include crescentic histiocytes, plasmacytoid monocytes, and extracellular debris.
    • Definitive diagnosis by FNA is uncommon; prudent pathologists are likely to report results as "suggestive of" or "compatible with" Kikuchi disease.
    • Confirm the diagnosis of Kikuchi disease by excisional biopsy in doubtful cases.
  • Excisional lymph node biopsy
    • Histologic findings consistent with Kikuchi disease
      • Paracortical necrosis may be patchy or confluent, and the degree of necrosis varies considerably from patient to patient.
      • Histocytes – Crescent-shaped nuclei (crescentic nuclei)
      • Other cells – Lymphocytes, plasmacytoid monocytes, macrophages, and immunoblasts (predominantly T cells)
      • Karyorrhexis – Histiocytes and macrophages containing phagocytized debris from degenerated lymphocytes
    • Absent or rare features in Kikuchi disease – Neutrophils, granulomas, plasma cells
Previous
Next

Histologic Findings

  • The 3 histologic phases of Kikuchi disease[6]
    • Proliferative phase – Initial phase with typical findings noted above
    • Necrotizing phase – Extensive necrosis that may destroy the normal architecture of the lymph node
    • Xanthomatous ("foamy cell") phase – The recovery phase with resolution of necrosis
  • Immunohistochemical studies
    • The immunophenotype of Kikuchi disease is primarily composed of mature CD8-positive and CD4-positive T lymphocytes. Lymphocytes and histiocytes also exhibit a high rate of apoptosis.
    • Relatively few B cells and natural killer (NK) cells are present.
    • Positive immunostaining results by monoclonal antibody Ki-M1P are seen in Kikuchi disease but not in malignant lymphoma.
  • Distinguishing Kikuchi disease from lymphoma
    • The numerous atypical monocytes and T-cell immunoblasts observed in Kikuchi disease may lead to an erroneous diagnosis of lymphoma, especially high-grade non-Hodgkin's lymphoma.
    • Features of Kikuchi disease that may help prevent its misdiagnosis as malignant lymphoma include the following:
      • Incomplete architectural effacement with patent sinuses
      • Presence of numerous reactive histiocytes
      • Relatively low mitotic rates
      • Absence of Reed-Sternberg cells
  • Distinguishing KD from SLE
    • Kikuchi disease and SLE have similar histopathologic appearances. Distinguishing the 2 entities can be difficult.
    • Kikuchi disease is suggested by the absence or paucity of the following:
      • Hematoxylin bodies
      • Plasma cells
      • Neutrophils
Previous
Proceed to Treatment & Management
 
 
Contributor Information and Disclosures
Author

John Boone, MD  Consulting Staff, Department of Otolaryngology, Naval Hospital Oak Harbor

John Boone, MD is a member of the following medical societies: American Academy of Otolaryngology-Head and Neck Surgery

Disclosure: Nothing to disclose.

Coauthor(s)

Charles S Kuzma, MD  Consulting Staff, Clinical Research Coordinator, First Health of the Carolinas Cancer Center

Charles S Kuzma, MD is a member of the following medical societies: American College of Physicians, American Society of Hematology, and California Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Karen Seiter, MD  Professor, Department of Internal Medicine, Division of Oncology/Hematology, New York Medical College

Karen Seiter, MD is a member of the following medical societies: American Association for Cancer Research, American College of Physicians, and American Society of Hematology

Disclosure: Novartis Honoraria Speaking and teaching; Novartis Consulting fee Speaking and teaching; Eisai Honoraria Speaking and teaching; Celgene Honoraria Speaking and teaching

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Lawrence H Brent, MD  Associate Professor of Medicine, Jefferson Medical College of Thomas Jefferson University; Chair, Program Director, Department of Medicine, Division of Rheumatology, Albert Einstein Medical Center

Lawrence H Brent, MD is a member of the following medical societies: American Association for the Advancement of Science, American Association of Immunologists, American College of Physicians, and American College of Rheumatology

Disclosure: Abbott Honoraria Speaking and teaching; Centocor Consulting fee Consulting; Genentech Grant/research funds Other; HGS/GSK Honoraria Speaking and teaching; Omnicare Consulting fee Consulting; Pfizer Honoraria Speaking and teaching; Roche Speaking and teaching; Savient Honoraria Speaking and teaching; UCB Honoraria Speaking and teaching

Rajalaxmi McKenna, MD, FACP  Southwest Medical Consultants, SC, Department of Medicine, Good Samaritan Hospital, Advocate Health Systems

Rajalaxmi McKenna, MD, FACP is a member of the following medical societies: American Society of Clinical Oncology, American Society of Hematology, and International Society on Thrombosis and Haemostasis

Disclosure: Nothing to disclose.

Chief Editor

Emmanuel C Besa, MD  Professor, Department of Medicine, Division of Hematologic Malignancies, Kimmel Cancer Center, Jefferson Medical College of Thomas Jefferson University

Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Clinical Oncology, American Society of Hematology, and New York Academy of Sciences

Disclosure: Nothing to disclose.

References

  1. Kampitak T. Fatal Kikuchi-Fujimoto disease associated with SLE and hemophagocytic syndrome: a case report. Clin Rheumatol. Aug 2008;27(8):1073-5. [Medline].

  2. Singh YP, Agarwal V, Krishnani N, Misra R. Enthesitis-related arthritis in Kikuchi-Fujimoto disease. Mod Rheumatol. May 10 2008;epub ahead of print. [Medline].

  3. Bosch X, Guilabert A, Miquel R, Campo E. Enigmatic Kikuchi-Fujimoto disease: a comprehensive review. Am J Clin Pathol. Jul 2004;122(1):141-52. [Medline]. [Full Text].

  4. Lin HC, Su CY, Huang CC, Hwang CF, Chien CY. Kikuchi's disease: a review and analysis of 61 cases. Otolaryngol Head Neck Surg. May 2003;128(5):650-3. [Medline].

  5. Baumgartner BJ, Helling ER. Kikuchi's disease: a case report and review of the literature. Ear Nose Throat J. May 2002;81(5):331-5. [Medline].

  6. Kuo TT. Kikuchi's disease (histiocytic necrotizing lymphadenitis). A clinicopathologic study of 79 cases with an analysis of histologic subtypes, immunohistology, and DNA ploidy. Am J Surg Pathol. Jul 1995;19(7):798-809. [Medline].

  7. Dorfman RF, Berry GJ. Kikuchi's histiocytic necrotizing lymphadenitis: an analysis of 108 cases with emphasis on differential diagnosis. Semin Diagn Pathol. Nov 1988;5(4):329-45. [Medline].

  8. Gallien S, Lagrange-Xelot M, Crabol Y, et al. [Systemic lupus erythematosus and Kikuchi-Fujimoto disease mimicking tuberculosis] [French]. Med Mal Infect. Jun 17 2008;epub ahead of print. [Medline].

  9. Yilmaz M, Camci C, Sari I, et al. Histiocytic necrotizing lymphadenitis (Kikuchi-Fujimoto's disease) mimicking systemic lupus erythematosus: a review of two cases. Lupus. 2006;15(6):384-7. [Medline].

  10. Poulose V, Chiam P, Poh WT. Kikuchi's disease: a Singapore case series. Singapore Med J. May 2005;46(5):229-32. [Medline]. [Full Text].

  11. Scagni P, Peisino MG, Bianchi M, et al. Kikuchi-Fujimoto disease is a rare cause of lymphadenopathy and fever of unknown origin in children: report of two cases and review of the literature. J Pediatr Hematol Oncol. Jun 2005;27(6):337-40. [Medline].

  12. Hedia G, Jamel A, Maher A, et al. Kikuchi-Fujimoto disease associated with systemic lupus erythematosus. J Clin Rheumatol. Dec 2005;11(6):341-2. [Medline].

  13. Onciu M, Medeiros LJ. Kikuchi-Fujimoto lymphadenitis. Adv Anat Pathol. Jul 2003;10(4):204-11. [Medline].

  14. Spies J, Foucar K, Thompson CT, LeBoit PE. The histopathology of cutaneous lesions of Kikuchi's disease (necrotizing lymphadenitis): a report of five cases. Am J Surg Pathol. Sep 1999;23(9):1040-7. [Medline].

  15. Sierra ML, Vegas E, Blanco-González JE, et al. Kikuchi's disease with multisystemic involvement and adverse reaction to drugs. Pediatrics. Aug 1999;104(2):e24. [Medline]. [Full Text].

  16. Lee S, Yoo JH, Lee SW. Kikuchi Disease: Differentiation from Tuberculous Lymphadenitis Based on Patterns of Nodal Necrosis on CT. AJNR Am J Neuroradiol. Dec 15 2011;[Medline].

  17. Hutchinson CB, Wang E. Kikuchi-Fujimoto disease. Arch Pathol Lab Med. Feb 2010;134(2):289-93. [Medline].

  18. Sopeña B, Rivera A, Vázquez-Triñanes C, Fluiters E, González-Carreró J, Del Pozo M, et al. Autoimmune Manifestations of Kikuchi Disease. Semin Arthritis Rheum. Dec 20 2011;[Medline].

  19. Hudnall SD. Kikuchi-Fujimoto disease. Is Epstein-Barr virus the culprit?. Am J Clin Pathol. Jun 2000;113(6):761-4. [Medline]. [Full Text].

  20. Atwater AR, Longley BJ, Aughenbaugh WD. Kikuchi's disease: case report and systematic review of cutaneous and histopathologic presentations. J Am Acad Dermatol. Jul 2008;59(1):130-6. [Medline].

  21. Yasukawa K, Matsumura T, Sato-Matsumura KC, et al. Kikuchi's disease and the skin: case report and review of the literature. Br J Dermatol. Apr 2001;144(4):885-9. [Medline].

  22. Sato Y, Kuno H, Oizumi K. Histiocytic necrotizing lymphadenitis (Kikuchi's disease) with aseptic meningitis. J Neurol Sci. Mar 1 1999;163(2):187-91. [Medline].

  23. Tong TR, Chan OW, Lee KC. Diagnosing Kikuchi disease on fine needle aspiration biopsy: a retrospective study of 44 cases diagnosed by cytology and 8 by histopathology. Acta Cytol. Nov-Dec 2001;45(6):953-7. [Medline].

  24. Viguer JM, Jiménez-Heffernan JA, Pérez P, et al. Fine-needle aspiration cytology of Kikuchi's lymphadenitis: a report of ten cases. Diagn Cytopathol. Oct 2001;25(4):220-4. [Medline].

  25. Kwon SY, Kim TK, Kim YS, et al. CT findings in Kikuchi disease: analysis of 96 cases. AJNR Am J Neuroradiol. Jun-Jul 2004;25(6):1099-102. [Medline]. [Full Text].

  26. Han HJ, Lim GY, Yeo DM, Chung NG. Kikuchi's disease in children: clinical manifestations and imaging features. J Korean Med Sci. Dec 2009;24(6):1105-9. [Medline]. [Full Text].

  27. Youk JH, Kim EK, Ko KH, Kim MJ. Sonographic features of axillary lymphadenopathy caused by Kikuchi disease. J Ultrasound Med. Jun 2008;27(6):847-53. [Medline].

  28. Jang YJ, Park KH, Seok HJ. Management of Kikuchi's disease using glucocorticoid. J Laryngol Otol. Sep 2000;114(9):709-11. [Medline].

  29. Hamdan AL, Jaber M, Tarazi A, Tawil A, Fuleihan N. Kikuchi Fujimoto disease: case report and review of the literature. Scand J Infect Dis. 2002;34(1):69-71. [Medline].

  30. Kishimoto K, Tate G, Kitamura T, Kojima M, Mitsuya T. Cytologic features and frequency of plasmacytoid dendritic cells in the lymph nodes of patients with histiocytic necrotizing lymphadenitis (Kikuchi-Fujimoto disease). Diagn Cytopathol. Nov 25 2009;epub ahead of print. [Medline].

  31. Lim GY, Cho B, Chung NG. Hemophagocytic lymphohistiocytosis preceded by Kikuchi disease in children. Pediatr Radiol. Jul 2008;38(7):756-61. [Medline].

  32. Ramirez AL, Johnson J, Murr AH. Kikuchi-Fujimoto's disease: an easily misdiagnosed clinical entity. Otolaryngol Head Neck Surg. Dec 2001;125(6):651-3. [Medline].

 
Previous
Next
 
 
 
 
All material on this website is protected by copyright, Copyright © 1994-2012 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.

DISCLAIMER: The content of this Website is not influenced by sponsors. The site is designed primarily for use by qualified physicians and other medical professionals. The information contained herein should NOT be used as a substitute for the advice of an appropriately qualified and licensed physician or other health care provider. The information provided here is for educational and informational purposes only. In no way should it be considered as offering medical advice. Please check with a physician if you suspect you are ill.