An unexpected asymptomatic epiglottal site of Kimura disease

Kimura disease (KD) is a chronic lymphoproliferative disorder of unknown etiology that affects the skin and lymph nodes, mostly observed in males of Asian descent. The natural history of asymptomatic epiglottal KD remains unknown. This rare site of KD is often only diagnosed when tumor growth starts to obstruct the upper airways.

A 34-year-old North African male presented with fatigue and multiple, slowly progressive, fluctuating skin nodules in the right mandibular and retroauricular regions. Computed tomography of the head and neck revealed a large soft tissue tumor close to the right mandibular body and unexpected thickening of the epiglottis. Transnasal laryngoscopy confirmed the CT findings and showed thickening of the epiglottis. The diagnosis of KD was based on histological examination of biopsy specimens taken from the right mandibular tumor, a cervical lymph node, and the epiglottis.

Most cases of KD with epiglottal involvement present with dysphonia and dysphagia. No consensus guidelines are available concerning the complementary investigations that should be performed. This case report raises the question of whether patients with suspected KD should be systematically screened for lesions in unusual and potentially dangerous anatomic sites.