Clinical Rheumatology

, 27:1073 | Cite as

Fatal Kikuchi–Fujimoto disease associated with SLE and hemophagocytic syndrome: a case report

Case Report


Kikuchi–Fujimoto disease (KFD) or histiocytic necrotizing lymphadenitis is a rare, benign, autoimmune condition characterized by lymphadenopathy, fever, and neutropenia. KFD can frequently mimic various diseases including infection, malignancy, and autoimmune disease. KFD has also been infrequently reported in association with SLE, which can be diagnosed previously, simultaneously, or after the diagnosis of SLE. The author presents here the first case of fatal KFD simultaneously occurred with SLE complicated with hemophagocytic syndrome and severe infection. The recognition of this association is of significance for management of affected patients with early intensive immunosuppressive therapy for favorable outcome.


Fatal Hemophagocytic syndrome Kikuchi–Fujimoto disease Lupus SLE 


  1. 1.
    Kucukardali Y, Somazgul E, Kunter E, Oncul O, Yildirim S, Kaplan M (2007) Kikuchi–Fujimoto disease: analysis of 244 cases. Clin Rheumatol 26:50–54PubMedCrossRefGoogle Scholar
  2. 2.
    Mahajan T, Merriman RC, Stone MJ (2007) Kikuchi–Fujimoto disease (histiocytic necrotizing lymphadentitis): report of a case with other autoimmune manifestations. Proc (Bayl Univ Med Cent) 20:149–151Google Scholar
  3. 3.
    Santana A, Lessa B, Galrao L, Lima I, Santiago M (2005) Kikuchi–Fujimoto’s disease associated with systemic lupus erythematosus: case report and review of the literature. Clin Rheumatol 24:60–63PubMedCrossRefGoogle Scholar
  4. 4.
    Quintas-Cardama A, Fraga M, Cozzi SN, Caparrini A, Maceiras F, Forteza J (2003) Fatal Kikuchi–Fujimoto disease: the lupus connection. Ann Hematol 82:186–188PubMedCrossRefGoogle Scholar
  5. 5.
    Bosch X, Guilabert A, Miquel R, Campo E (2004) Enigmatic Kikuchi–Fujimoto disease: a comprehensive review. Am J Clin Pathol 122:141–152PubMedCrossRefGoogle Scholar
  6. 6.
    Bosch X, Guilabert A (2006) Kikuchi–Fujimoto disease. Orphanet J Rare Dis 1:18PubMedCrossRefGoogle Scholar
  7. 7.
    Larroche C, Mouthon L (2004) Pathogenesis of hemophagocytic syndrome (HPS). Autoimmune Rev 3:69–75CrossRefGoogle Scholar
  8. 8.
    Romanou V, Hatzinikolaou P, Mavragani KI, Meletis J, Vaiopoulos G (2006) Lupus erythematosus complicated by hemophagocytic syndrome. J Clin Rheumatol 12:301–303PubMedCrossRefGoogle Scholar
  9. 9.
    Qian J, Yang CD (2007) Hemophagocytic syndrome as one of main manifestations in untreated systemic lupus erythematosus: two case reports and literature review. Clin Rheumatol 26:807–810PubMedCrossRefGoogle Scholar

Copyright information

© Clinical Rheumatology 2008

Authors and Affiliations

  1. 1.Division of Allergy and Clinical Immunology, Department of Internal Medicine, Faculty of MedicineChulalongkorn University HospitalBangkokThailand

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