Background: Angiolymphoid hyperplasia with eosinophilia (ALHE) and Kimura's Disease (KD) share many clinical and histopathological features. Although they were once considered different stages of the same disease, they are now known to represent separate entities. Recently, ALHE is being called epithelioid hemangioma (EH), a term that better describes the possible neoplastic nature of the entity. Case Presentation: An eighteen year-old Asian female presented with a three-month history of fluctuating swelling and ptosis of the left upper eyelid. Computed tomography disclosed a distinct homogeneous lesion in the left superior orbit, molding to the globe and other orbital structures. At histopathological evaluation the lesion was composed of numerous blood vessels lined by plump endothelial cells with oval nuclei protruding into the lumen. Surrounding the vessels, there was a chronic inflammatory infiltrate with a large proportion of eosinophils. Based on clinical and histopathological findings, the diagnosis of EH was made. Conclusion: Although exams like blood count, urinalysis and whole body scans can assist in the differential diagnosis, EH can be diagnosed and differentiated from KD on histopathological grounds. The presence of vascular hyperplasia with plump endothelial cells protruding into the lumen is the most important discriminator in establishing the diagnosis of EH. Such distinction is crucial for the patient because EH is not associated with any of the systemic manifestations present in KD.
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