Angiolymphoid Hyperplasia with Eosinophilia of the Orbit and Adnexa

Purpose: Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare disorder presenting with solitary or multiple nodules in the dermis or subcutaneous tissues. ALHE shares clinical as well as histopathological characteristics with Kimura disease (KD), but they have been considered to be two distinct entities based on their histological features. Orbital and adnexal involvement in ALHE is rare. The published literature is limited to few case reports featuring single cases. Methods: We report a series of 5 cases of ALHE presenting with diverse clinical features seen at a tertiary referral care centre in India. We also review the published literature with a special emphasis on the treatment modalities for orbital and adnexal ALHE. Results: Three patients of this series presented with orbital involvement, while the remaining 2 had involvement of the eyelid. Three patients underwent incisional and/or excisional biopsy, whereas 2 were managed conservatively. There were no recurrences seen on follow-up. Conclusions: ALHE can rarely involve the orbit and adnexa. There is no consensus on the best modality of management of this rare entity. ALHE and KD have often been considered variations of the same disease, but it is important to differentiate between the two entities for optimal patient management.