A case of angiolymphoid hyperplasia with eosinophilia

A 43-year old woman presented with an 18 months history of clustered purple papules on the concha of her left ear, adjacent to the external auditory meatus.
The papules were slowly increasing in size and were itchy and prone to bleeding. There was no improvement in symptoms with topical steroids. Punch biopsy of one of the lesions showed a nodular appearing proliferation in the dermis with a diffuse inflammatory component together with variably sized and shaped vascular spaces, lined by plump-appearing endothelial cells. The inflammatory component consisted of numerous eosinophils, lymphocytes and histiocytes. This was consistent with a diagnosis of angiolymphoid hyperplasia with eosinophilia (ALHE). A full blood count was performed and eosinophils were within the normal range.
ALHE is an uncommon, benign vasoproliferative disorder of unclear origin. It is characterised by a proliferation of histiocytoid endothelial cells with prominent lymphocytic and eosinophilic infiltration. It typically presents with solitary or multiple pink to red-brown dome shaped papules. The ear and forehead are involved in 50% of patients. Lesions can vary from asymptomatic to painful, pruritic lesions which may bleed. Treatment is often pursued to provide symptomatic relief and address cosmetic concerns.
A recent systematic review analysing 908 patients with this condition noted that topical and systemic corticosteroids are almost wholly ineffective for ALHE and that excision still remains the best treatment option. Unfortunately this treatment modality is also sub optimal with a recurrence rate of approximately 40%.1
1. Adler, Brandon L. et al. "Epidemiology and Treatment of Angiolymphoid Hyperplasia with Eosinophilia (ALHE): A Systematic Review". Journal of the American Academy of Dermatology 74.3 (2016): 506-512.e11. Web.

Dr. Leonard Timoney