Kikuchi-Fujimoto Disease - A Clinical Enigma: Rare two Case Reports

  • Jayanti Khura Senior Resident, Department of Medicine, PGIMER and Dr. RML Hospital, New Delhi, India.
  • Shalini Shakarwal Ex-Assistant Professor, Department of Obs. & Gynae.
  • Vijay Kumar Professor, Department of Pathology, ABVIMS & Dr. RML Hospital, New Delhi, India.
  • Sanjay Kumar Associate Professor, Department of Medicine, Lady Hardinge Medical College, New Delhi, India.
  • Pratap Singh Associate Professor, Department of Medicine, PGIMER and Dr. RML Hospital, New Delhi, India. https://orcid.org/0000-0001-6291-4287
Keywords: Kikuchi-Fujimoto Disease, KFD, Histiocytic Necrotizing Lymphadenitis, Lymphadenopathy, Systemic Lupus Erythematosus, SLE

Abstract

Background: Kikuchi-Fujimoto Disease (KFD) is a rare disease and is commonly seen in Asian population. It usually presents with prolonged fever, leukopenia and persistent cervical lymphadenopathy. It is a benign disease with a female preponderance. The cause is unknown but many theories have been postulated, like autoimmune, inflammatory, infectious agents and molecular mimicry. Most of the patients will recover themselves without any sequelae, in six-month time. However, it may rarely also convert to autoimmune disease, like SLE. The recurrence rate is very low. It is important to consider it as a differential diagnosis in chronic persistent lymphadenopathy, like tuberculosis, lymphoma, HIV and autoimmune lymphadenitis.

Cases: Here we present a case of a 37-year-old female who initially went to a private practitioner with chief complaint of a persistent neck mass of approximately 6 months duration, multiple small joints pain and persistent fever. She underwent excisional biopsy for suspected lymphoma, but final pathology rendered a diagnosis of KFD. Second case also presented with multiple neck swelling and joint pains, suspect of SLE, connective tissue disorder or young-onset still’s disease was made clinically but excisional biopsy revealed KFD. Both the patients improved with NSAIDS and low dose wysolone therapy.

Conclusion: The purpose of this article is not only to review the literature but also to create awareness of this entity in the differential diagnosis of persistent lymphadenopathy, especially for the general otolaryngologist in a community-based setting. In addition, this review would be beneficial for other practitioners as well, specifically paediatricians, infectious disease physicians, rheumatologists, pathologists, and medical oncologists.

How to cite this article:
Khura J, Shakarwal S, Kumar V, Kumar S, Singh P. Kikuchi-Fujimoto Disease - A Clinical Enigma: Rare two Case Reports. J Adv Res Med 2019; 6(3): 12-15.

DOI: https://doi.org/10.24321/2349.7181.201914

References

Debley JS, Rozansky DJ, Miller ML, Katz BZ, Greene ME. Histiocytic necrotizing lymphadenitis with autoimmune

phenomena and meningitis in a 14 years girl. Pediatrics 1996; 98(1): 30-33. Available from: https://pediatrics.

aappublications.org/content/98/1/130 [PubMed/ Google Scholar].

Deaver D, Naghashpour M, Sokol L. Kikuchi-Fujimoto disease in the United States: three case reports and

review of the literature. Mediterr J Hematol Infect Dis 2014; 6(1): e2014001. [PubMed/ Google Scholar].

Bosch X, Guilabert A. Kikuchi-Fujimoto disease. Orphanet J Rare Dis 2006; 1: 18. Available from: https://ojrd.

biomedcentral.com/articles/10.1186/1750-1172-1-18 [Google Scholar].

Qadri F, Atkin GK, Thomas D, Das SK. Kikuchi’s disease: an important cause of cervical lymphadenopathy. Clin

Med 2007; 7: 82-84. [PubMed/ Google Scholar].

Santana A, Lessa B, Glarao L, Lima I, Santiago M. Kikuchi-Fujimoto’s disease associated with systemic

lupus erythematosus: case report and review of the literature. Clin Rheumatol 2005; 24(1): 60-63. [PubMed/

Google Scholar].

Lee BC, Patel R. Kikuchi-Fujimoto disease: a 15-year analysis at a children’s hospital in the United States.

Clin Pediatr 2013; 52(1): 92-95. Available from: https://utsouthwestern.pure.elsevier.com/en/publications/

kikuchi-fujimoto-disease-a-15-year-analysis-at-achildrens-hospit [Google Scholar].

Poulouse V, Chiam P, Poh W. Kikuchi’s disease: a Singapore case series. Singapore Med J 2005; 46: 229-

[PubMed/ Google Scholar].

Bosch X, Guilabert A, Miquel R, Campo E. Enigmatic Kikuchi-Fujimoto disease: a comprehensive review.

Am J Clin Pathol 2004; 122(1): 141-152. [PubMed/ Google Scholar].

Feder HM, Liu J, Rezuke WN. Kikuchi disease in Connecticut. J Pediatr 2014; 164(1): 196-200. [PubMed/

Google Scholar].

Kuculardali Y, Solmazgul E, Kunter E, Oncul O, Yildirim S, Kaplan M. Kikuchi-Fujimoto disease: analysis of 244

cases. Clin Rheumatol 2007; 26(1): 50-54. Available from: https://link.springer.com/article/10.1007/

s10067-006-0230-5 [PubMed/ Google Scholar].

Baenas DF, Diehl FA, Salinas MH, Riva C, Diller A, Lemos P. Kikuchi-Fujimoto disease and systemic lupus

erythematosus. Int Med Case Rep J 2016; 9: 163-167. [PubMed/ Google Scholar].

Goldblatt F, Andrews J, Russell A, Isenberg D. Association of Kikuchi-Fujimoto’s disease with SLE. Rheumatology 2008; 47(4): 553-554. Available from: https://academic.oup.com/rheumatology/article/47/4/553/1790090 [PubMed/ Google Scholar].

Londhey VA, Buche AS, Kini SH, Rajadhyaksha GC. Kikuchi fujimoto disease and systemic lupus erythematosus-a rare association. The Journal of the Association of Physicians of India 2010; 58: 642-643. [PubMed/ Google Scholar].

Published
2020-02-25