Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2018 May 25;5(5):000856.
doi: 10.12890/2018_000856. eCollection 2018.

Kikuchi-Fujimoto Disease Associated with Sjögren's Syndrome: A Case Report

Abdul-Wahab Al-Allaf  1 , Yousef M Yahia  1
Affiliations

Kikuchi-Fujimoto Disease Associated with Sjögren's Syndrome: A Case Report

Abdul-Wahab Al-Allaf et al. Eur J Case Rep Intern Med. .

Abstract

Kikuchi-Fujimoto disease (KFD) or Kikuchi histiocytic necrotizing lymphadenitis, is an extremely rare, benign and self-limiting disease which can be confused with lymphoma and sometime with infections such as tuberculosis. It has also been infrequently reported in association with autoimmune diseases. KFD shares sex and age predisposition as well as histological features with systemic lupus erythematosus. The co-existence of KFD with primary Sjögren's syndrome has only been rarely reported. Here we describe the case of a 33-year-old woman who was diagnosed with KFD and also found to have primary Sjögren's syndrome.

Learning points: To understand the relationship between Kikuchi-Fujimoto disease (KFD) and Sjögren's syndrome.KFD shares some characteristics with autoimmune diseases.It is important to maintain a high index of suspicion of Sjögren's syndrome and diffuse lymphadenopathy in patients with KFD.

Keywords: Kikuchi-Fujimoto disease; Sjögren’s syndrome; necrotizing histiocytic lymphadenitis.

PubMed Disclaimer

Conflict of interest statement

Conflicts of Interests: The Authors declare that there are no competing interests.

Figures

Figure 1
Figure 1
Histopathology of the LN biopsy showing central ncerotizing granulomatous areas, surrounded with giant cells
See this image and copyright information in PMC

References

    1. Zhang J, Yang J, Weng W-W, Zhu Y-J, Qiu H, Dong M-J. Kikuchi-Fujimoto disease associated with Sjogren’s syndrome: a case report and review of the literature. Int J Clin Exp Med. 2015;8:17061–17066. - PMC - PubMed
    1. Asano S, Akaike Y, Jinnouchi H, Muramatsu T, Wakasa H. Necrotizing lymphadenitis: a review of clinicopathological, immunohistochemical and ultrastructural studies. Hematol Oncol. 1990;8:251–260. - PubMed
    1. Dorfman RF, Berry GJ. Kikuchi’s histiocytic necrotizing lymphadenitis: an analysis of 108 cases with emphasis on differential diagnosis. Semin Diagn Pathol. 1988;5:329–345. - PubMed
    1. Bosch X, Guilabert A, Miquel R, Campo E. Enigmatic Kikuchi-Fujimoto disease: a comprehensive review. Am J Clin Pathol. 2004;122:141–152. - PubMed
    1. Miyashita Y, Yamaguchi M, Fujimoto W. Painful indurated erythema suggestive of Kikuchi-Fujimoto disease in a patient with primary Sjögren’s syndrome. J Dermatol. 2003;30:608–611. - PubMed

LinkOut - more resources