Kimura Disease of the Epiglottis: A Case Report and Review of Literature

Abstract

Kimura disease (KD) affecting an unusual site is a diagnostic challenge. We report herein the case of a 62-yearold Japanese woman who presented with swelling of the epiglottis, resulting in airway narrowing. Microscopically, biopsied and resected specimens both revealed lymphoid proliferation of a reactive immunophenotype, accompanied by vascular proliferation, eosinophilic infiltration, and stromal sclerosis. Adjunctive immunohistochemistry with immunoglobulin E in addition to laboratory and histological findings led us to seriously consider a diagnosis of KD. The patient underwent surgical removal with postoperative steroid therapy and has no evidence of recurrence. Our experience suggests that KD is potentially fatal as well as showing difficulty in the histological diagnosis when occurring in the upper respiratory tract, such as the epiglottis. A literature review disclosed that our case is the 11th case so far reported in this location, and that KD of the epiglottis did not show any male preponderance, as seen in other places.

Co-existence of lip and epiglottis Kimura’s disease

“… 7 Similarly, KD of the epiglottis is also extremely uncommon; only 11 cases have been reported in the literature. 8 Symptoms were mostly related to airway narrowing, and only 2 patients were found asymptomatic, which is similar to our case. 7 Further, the co-existence of the lip and epiglottic KD is not previously reported.…”

“…The pathogenesis of KD is not well known; however, allergic reaction or an alteration of immune regulation can be the possible cause. 8 Surgical resection is the standard treatment option for KD. Adjuvant therapy in the form of steroids, cytotoxic therapy, and radiation therapy also have been used in some cases.…”

Concurrent Kimura disease and lupus nephritis

“…[ 12 – 14 ] As in this patient, almost all patients have marked IgE elevations and peripheral eosinophilia, and the percentage of eosinophils is as high as 10% to 20%. [ 15 , 16 ] Diagnosis of the disease relies on pathologic study of lymph node or subcutaneous nodule biopsies demonstrating follicle-like growth and infiltration by eosinophils, which frequently form eosinophilic microabscess. [ 17 ] The current case had recurrent episodes of lymphadenopathy over more than 40 years with peripheral eosinophilia and marked IgE elevations, and pathologic examination of excised lymph nodes suggested eosinophil infiltration and eosinophilic granuloma.…”

Synchronous Kimura lesions at two different sites–a diagnostic dilemma!

“…Other sites of involvement include the groin (15%), extremities (12%) and trunk (3%). Unusual sites of involvement include the epiglottis, lacrimal glands, tympanic membranes, paranasal sinuses, parapharyngeal spaces, colon, spermatic cord and the median nerve (9,11,(13)(14)(15). While systemic symptoms are uncommon, renal involvement is often seen (10-60%).…”