Angiolymphoid hyperplasia with eosinophilia (ALHE) of orbit, a case report

Angiolymphoid hyperplasia with eosinophilia (ALHE) is an uncommon idiopathic condition thatmanifests in adults as isolated or grouped papules, plaques, or nodules in the skin of the headand neck. Most patients present with lesions in the periauricular region, forehead, or scalp.Rare sites of involvementinclude the hands, shoulders, breasts, penis, oral mucosa, and orbit.A distinct pathologic entity, ALHE is marked by a proliferation of blood vessels withdistinctive large endothelial cells. These blood vessels are accompanied by a characteristicinflammatory infiltrate that includes eosinophils. The lesion is benign but may be persistentand is difficult to eradicate. Whether ALHE represents a benign neoplasm or an unusualreaction to varied stimuli, including trauma, remains unclear.More new information suggest that ALHE may be a primary lymphoproliferative process, asevidenced by findings of T-cell genetic factor rearrangements, although PCR analysis has notconfirmed monoclonality in all cases.Angiolymphoid hyperplasia with eosinophilia (ALHE (plus Kimura s Disease (KD) sharemany clinical plus histopathological features. Although they were once careful differentstages of the same disease, they are now known to represent separate entities.A 39-year-old patient is reported with a 1 cm well circumbscribed mass in the supranasalregion of right orbir with clinically diagnosis of schwannoma. Histological examination ofthe excised mass disclosed angiolymphoid hyperplasia with eosinophilia.Characteristic histologic features of ALH present in thiscase contained within propagation ofthick-walled blood vessels wrinkled by prominent endothelial cells, infiltration of theinterstitium by chronic provocative cells (mostly eosinophils), plus presence of lymphoidfollicles with germinal centers.