Enigmatic Kikuchi-Fujimoto Disease: A Comprehensive Review

Xavier Bosch, MD; Antonio Guilabert, MD; Rosa Miquel, MD; Elias Campo, MD

Disclosures

Am J Clin Pathol. 2004;122(1) 

In This Article

Abstract and Introduction

Abstract

To determine the clinicopathologic significance of Kikuchi-Fujimoto disease (KFD) and review the literature on this condition, we conducted a MEDLINE search of English-language articles published between 1972 and December 2003.

KFD has a worldwide distribution, and Asiatic people have a higher prevalence. Its pathogenesis remains controversial. Patients are young and seek care because of acute tender, cervical lymphadenopathy and low-grade fever. Histologic findings include paracortical areas of coagulative necrosis with abundant karyorrhectic debris. Karyorrhectic foci consist of various types of histiocytes, plasmacytoid monocytes, immunoblasts, and small and large lymphocytes. There is an abundance of T cells with predominance of CD8+ over CD4+ T cells. Differential diagnosis includes lymphoma, lymphadenitis associated with systemic lupus erythematosus, and even adenocarcinoma. KFD is an uncommon, self-limited, and perhaps underdiagnosed process with an excellent prognosis. Accurate clinicopathologic recognition is crucial, particularly because KFD can be mistaken for malignant lymphoma.

Introduction

Kikuchi-Fujimoto disease (KFD; so-called histiocytic necrotizing lymphadenitis) is an enigmatic, benign, and self-limited syndrome characterized by regional lymphadenopathy with tenderness, usually accompanied by mild fever and night sweats. Initially described in Japan, KFD was first reported almost simultaneously by Kikuchi and by Fujimoto and associates in 1972 as a lymphadenitis with focal proliferation of reticular cells accompanied by numerous histiocytes and extensive nuclear debris.[1] Clinicians and pathologists are unfamiliar with this entity. We provide a comprehensive review of KFD with special emphasis on the clinicopathologic significance of this condition.

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