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Kang, Choi, Jung, Son, Lee, Yang, Park, Lee, and Lee: Kikuchi-Fujimoto's Disease with Adult Onset Still's Disease
Original Article

J Korean Rheum Assoc 2008;15(1):58-62.

Published online: 20 March 2008

DOI: https://doi.org/10.4078/jkra.2008.15.1.58

Kikuchi-Fujimoto's Disease with Adult Onset Still's Disease

Eun-Jin Kang, M.D., Sang Tae Choi, M.D., Sang Youn Jung, M.D., Myoung Kyun Son, M.D, Kwang-Hoon Lee, M.D., Woo Ick Yang, M.D.∗, Yong Beom Park, M.D., Soo-Kon Lee, M.D., Sang-Won Lee, M.D.

Division of Rheumatology, Department of Internal Medicine, Seoul, Korea

∗Department of Pathology, Yonsei University College of Medicine, Seoul, Korea

Received 29 October 2007       Accepted 29 November 2007

Copyright © 2008 The Korean Rheumatism Association

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Kikuchi-Fujimoto's disease (KFD), or histiocytic necrotizing lymphadenitis, is a rare benign and self-limiting disease. KFD are confused with systemic autoimmune disease as they present with localized lymphadenopathy, fever, fatigue, arthritis, leukopenia. Furthermore as KFD can occur associated with other autoimmune disease, we need to diagnose carefully. Here, we describe a case of 27-year-old female patient, diagnosed as KFD, who subsequently developed adult onset Still's disesase (AOSD). As far as we know, this is the first case of KFD with AOSD in Korea.

Keywords: Kikuchi-Fujimoto's disease, AOSD, Lymphadenopathy

References

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Fig. 1.
Neck computed tomography scan shows mild hyperplasia of lingular tonsils (arrow head) and multiple lymphadenopathies (arrow) bilaterally.
jkra-15-58f1.tif
Fig. 2.
Lymph node biopsy shows necrotizing lesion composed of various histiocytes, plasmacytoid monocytes and lymphoid cells with karyorrhectic nuclear debris (H&E, original magnification ×200).
jkra-15-58f2.tif
Fig. 3.
Changes of liver function tests and serum ferritin level during the course of treatment. LDH: lactate dehydrogenase, AST: aspartate aminotransferase, ALT: alanine aminotransferase.
jkra-15-58f3.tif
Table 1.
Cases of Kikuchi-Fujimoto's disease (KFD) with adult onset Still's disease (AOSD)
Reference Sex/age Diagnosis of AOSD Lymphadenopathy Treatment Prognosis
Ohta et al (7) M/41 Arthralgia, fever, rash, sore throat, abnormal LFT, splenomegaly Cervical, axillary inguinal NSAID prednisolone (50 mg/day) Improved
  M/19 Arthralgia, fever, leukocytosis, abnormal LFT, hepatosplenomegaly Cervical NSAID prednisolone Improved
  F/14 Arthralgia, fever, rash, leukocytosis, sore throat, hepatomegaly Cervical Prednisolone (30 mg/day) Improved
Lyberatos et al (14) M/18 Arthralgia, fever, rash, leukocytosis, abnormal LFT, hepatosplenomegay Generalized Prednisolone Improved
  F/47 Arthralgia, fever, rash, sore throat Cervical, axillary Aspirin Improved
Cousin et al (15) M/32 Arthralgia, fever, rash, leukocytosis, ANA(−), RF(−) Cervical, axillary inguinal Prednisolone (60 mg/day) Improved
Our patient F/27 Arthralgia, fever, rash, leukocytosis, sore throat, abnormal LFT, ANA(＋) Cervical Prednisolone (60 mg/day) Improved
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