Angiolymphoid hyperplasia with eosinophilia (ALHE) and Kimura's Disease (KD) share many clinical and histopathological features. [1] Although they were once considered different stages of the same disease, they are now known to represent separate entities. [2] Recently, ALHE is being called epithelioid hemangioma (EH), a term that better describes the most distinguish feature of this entity: the abnormal proliferation of endothelial cells. [3]

EH usually presents as small, red, pruritic plaques in the subcutis or dermis of the head and neck region. Orbital involvement in EH is a relatively rare manifestation of the disease with only scattered case reports published in literature. [4]

Epithelioid hemangioma (EH) and Kimura's Disease (KD) share many clinical and histopathological features [1] Although they were once considered different stages of the same disease, they are now known to represent separate entities [2] The former is a localized hyperplasia of atypical endothelial cells with no systemic involvement. On the other hand, the latter can course with lymphadenopathy, blood eosinophilia, and nephrotic syndrome due to IgE deposition in the renal glomeruli [1]

EH was first described in 1969 [5] It presents as nodules or erythematous subcutaneous papules, usually in the head and neck region of young women [6] It can occur in all races. Whenever the orbit is involved, common symptoms are proptosis, tearing, pruritus around the eye, and blurred peripheral vision. [4] The case presented herein had no associated symptoms besides the swelling of the eyelid, which makes the presentation even more atypical. Histologically, most lesions are well-circumscribed and composed of vessels lined by plump endothelial cells that protrude into the lumen in a "tombstone fashion" [7] Surrounding the vessels, there is usually a prominent inflammatory infiltrate. A large proportion of eosinophils can often be seen.

KD probably represents an allergic or autoimmune response that typically presents as subcutaneous nodules in the head and neck region of young Asian males [6] Systemic associations include blood eosinophilia, nephrotic syndrome due to IgE depostion in the renal glomeruli, lymphadenopathy and, less common, asthma, tuberculosis and Loffler syndrome.[1]

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.