Kimura Disease

Updated: Sep 27, 2016
  • Author: Evan W Piette, MD; Chief Editor: William D James, MD  more...
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Overview

Background

Kimura disease is a chronic inflammatory disorder of unknown etiology that most commonly presents as painless lymphadenopathy or subcutaneous masses in the head and neck region. The first report of Kimura disease was from China in 1937, in which Kimm and Szeto [1] described seven cases of a condition they termed "eosinophilic hyperplastic lymphogranuloma." The disorder received its current name in 1948, when Kimura et al [2] noted the vascular component and referred to it as an "unusual granulation combined with hyperplastic changes in lymphoid tissue."

Controversy has existed in the literature regarding whether Kimura disease and angiolymphoid hyperplasia with eosinophilia (ALHE) are the same entity. Some authors believe that Kimura disease represents a chronic, deeper form of ALHE; however, most recent papers distinguish the two on the basis of clinical and histopathologic characteristics. [3, 4] ALHE appears to represent an arteriovenous malformation with secondary inflammation. Kimura disease may represent a primary inflammatory process with secondary vascular proliferation.

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Pathophysiology

The pathophysiology of Kimura disease remains unknown. It has been hypothesized that an infection or toxin may trigger an autoimmune phenomenon or lead to a type I (immunoglobulin E [IgE]–mediated) hypersensitivity reaction. Some evidence has suggested a predominance of TH 2 cells in patients with Kimura disease. [5] Additional studies have shown elevated granulocyte macrophage-stimulating factor (GM-CSF), tumor necrosis factor-a (TNF-a), soluble IL-2 receptor (sIL-2R), IL-4, IL-5, IL-10, and IL-13. [6, 7] These findings may help lay the groundwork for elucidating the underlying pathophysiology of Kimura disease.

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Epidemiology

Frequency

United States

Kimura disease has rarely been reported in the United States.

International

The exact prevalence of Kimura disease is not known. Most cases of this rare disease are reported in East and Southeast Asia, with a small number of cases reported in Europe. [8]

Race

Most cases of Kimura disease have been reported in Asians, and the prevalence among persons of other races is thought to be low. A retrospective review of 21 histopathologic specimens diagnosed as Kimura disease at the US Armed Forces Institute of Pathology found the following racial distribution: 7 whites, 6 African Americans, 6 Asians, 1 Hispanic, and 1 Arab. [9] This illustrates that if clinically suspected, Kimura disease should be included on the differential diagnosis for persons of any racial group. [10]

Sex

Males are affected by Kimura disease more commonly than females, with a 3.5:1 to 9:1 male-to-female ratio in most series reported, with the exception of one series in which the male-to-female ratio was 19:1. [10, 11]

Age

Kimura disease is usually seen in young adults during the third decade of life, with the median age being 28-32 years. [9, 12, 13]

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Prognosis

The course of Kimura disease is chronic, with lesions frequently persisting or recurring despite treatment. Kimura disease can lead to disfigurement secondary to the growth of untreated lesions, particularly given the predilection for the head and neck. Additionally, recurrence after treatment is well described. To date, malignant transformation of Kimura disease has not been described in the literature.

 

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