Rare case of Kimura disease of the upper lip: A case report

Abstract

The main symptom of Kimura disease (KD), a rare, chronic inflammatory disorder of unknown etiology, is painless granulation preferentially developing in the head and neck region’s soft tissues, especially in the parotid and submandibular glands and neck lymph nodes. KD is characterized clinically by peripheral blood eosinophilia and elevated serum immunoglobulin E (IgE) levels and histopathologically by high capillary vascularization and lymphoid follicle formation with eosinophil infiltration in the connective tissues. Angiolymphoid hyperplasia with eosinophilia (ALHE), a rare benign tumor of unknown etiology, also occurs in the head and neck frequently as single/multiple angiomatous papules/nodules varying from light brown to pink. Histopathologically, accelerated endothelial cell proliferation and high lymphocyte and eosinophil infiltration are observed. Due to KD’s and ALHE’s similar clinical and pathological symptoms, differential diagnosis is challenging. Their manifestations include a painless head/neck subcutaneous swelling. Here, we have described a case involving a 44-year-old man who presented with an upper lip mass that increased in size for 1 year. Laboratory studies revealed leukocytosis and eosinophilia. Computed tomography and magnetic resonance imaging examinations revealed a submucosal lip mass. Suspect minor salivary gland malignancies and lymphomas were surgically excised. Histopathologically, we observed marked lymphoid follicle proliferation with inflammatory cell, including eosinophil infiltration, capillary proliferation, and a developed vessel not normally present in the lip. Because differentiating between KD and ALHE is difficult, clinical differentiation was our focus. The Asian male patient with lymphadenopathy, eosinophilia, and high IgE levels was finally diagnosed with KD that has not recurred in 1 year.