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      Drugs & Diseases > Dermatology

      Kimura Disease

      Updated: Mar 15, 2022
      • Author: Alan Snyder; Chief Editor: William D James, MD  more...
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      Overview

      Practice Essentials

      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 7 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 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. [5, 6]  Reports have described both diseases presenting simultaneously. [7]

      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. [8]  Additional studies have shown elevated granulocyte-macrophage stimulating-factor (GM-CSF), tumor necrosis factor-α (TNF-α), soluble interleukin (IL)–2 receptor (sIL-2R), IL-4, IL-5, IL-10, and IL-13. [9, 10]  Another study indicated that the activation of the IL-21/pERK1/2 pathway is a component of Kimura disease immunopathogenesis and that pERK1/2 could be a potential prognostic indicator of the disease. [11]  These findings may help lay the groundwork for elucidating the underlying pathophysiology of Kimura disease.

      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. Smoking habits and a history of systemic disease also have been associated with poor prognosis of the disease. [12]  To date, malignant transformation of Kimura disease has not been described in the literature.

      Complications

      Rarely, large nodules or tumors from Kimura disease have ulcerated. After treatment, recurrence also has been reported.

      Abbas et al reported 1 case of postsurgical facial disfigurement effectively treated with photodynamic therapy. [13]

      On 2 known occasions, brain embolisms have occurred due to Kimura disease–induced thrombi. [14]

      Diagnostics

      Nearly all patients with Kimura disease demonstrate peripheral eosinophilia and elevated levels of serum IgE. In 1 series, the number of eosinophils was closely correlated to the sizes of the neck masses. [15]

      Blood urea nitrogen, creatinine, and urinary protein levels should be obtained to exclude concomitant renal dysfunction (especially nephrotic syndrome).

      Serum eosinophil cationic protein levels parallel the course of the disease. [16]

      Incisional biopsy is recommended to obtain the diagnosis of Kimura disease.

      Treatment

      Conservative surgical excision has been considered the treatment of choice for Kimura disease. [17, 18]  Recurrence after surgery is frequently observed.

      Consultations

      Consultation with an otolaryngologist or ophthalmologist should be considered for further evaluation depending on the extent and location of the disease.

      Next:

      Epidemiology

      Kimura disease has rarely been reported in the United States. 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 and the Middle East. [19, 20]

      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. [21] This illustrates that if clinically suspected, Kimura disease should be included on the differential diagnosis for persons of any racial group. [22]

      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. [22, 23]  Kimura disease is usually seen in young adults during the third decade of life, with the median age being 28-32 years. [21, 24, 25]  The disease can also occur in older patients. [26, 27] Although more rare, pediatric populations can develop Kimura disease, and cases have been reported in persons aged as young as 15 months. [20]

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