Kimura Disease: Rare Manifestations in Unusual Populations

Kimura disease (KD) is a benign, rare, and chronic inflammatory disorder with an angiolymphatic proliferation of unknown etiology involving subcutaneous tissues, affecting a population sporadically. It is most common in middle-aged Asian men, as it is endemic in china and Japan. Herein, we present a medically free 40 years old Saudi female, with unusual clinical, radiological and histopathological manifestations of KD presented with extended facial swelling from the right to the left cheek with a bluish and firm mass in the center of the hard palate. Additionally, a solitary soft lymph node swelling was found in the anterior triangle of the neck. CT scan of the head and neck showed an area of nodularity and fat stranding in the left lower cheek. Histologically, hyperplastic changes of lymphatic tissues were viewed. Treatment with prednisolone showed transient improvement, thus Cyclosporine was suggested since it has a significant potential in decreasing the recurrences and the flare-ups of KD. Adequate reporting and documentation of those cases in the literature are strongly required to establish clear guidelines for the diagnosis and management of KD among Middle eastern population.