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-year-old 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.

Fig. 1

Endoscopic view showing a mass-like swelling of the epiglottis about to obstruct the airway

Fig. 2

CT showing a mass-like thickening of the epiglottis (a), and multiple cervical lymph node enlargement (b). T1-weighted (c) and T2-weighted (d) MRI displaying a homogenously hyperintense mass in the epiglottis

Fig. 3

Resected specimen showing submucosal nodular lymphoid aggregates containing follicles and germinal centers with sclerotic stroma (hematoxylin–eosin, original magnification ×20)

Fig. 4

Increased vessels and inflammatory infiltrates within and around the lymphoid follicles (hematoxylin–eosin, original magnification ×50)

Fig. 5

Increased vessels and prominent eosinophils (hematoxylin–eosin, original magnification ×400)

Fig. 6

Proteinaceous deposits in a germinal center (hematoxylin–eosin, original magnification ×400)

Fig. 7

Germinal centers showing reticular staining of IgE and surrounding IgE-positive mast cells (original magnification ×100)