Unusual Merkel cell carcinoma of the eyelid

Merkel cell carcinoma of the eyelid is a rare tumor with less than 100 reported cases worldwide. We describe an unusual presentation of Merkel cell carcinoma of the eyelid in a 60 year old Asian male. He presented with multiple left lower lid conjunctival nodules, intense conjunctival erythema, as well as ipsilateral cervical lymphadenopathy. An incisional biopsy diagnosed him with Merkel cell carcinoma with a PET scan showing distant metastatic disease. He was then treated with chemotherapy. The combination of a presentation of conjunctival nodules and erythema, location in the lower eyelid and the conjunctiva, the presence of metastatic disease on diagnosis as well as an unusual immunohistochemical profile make this an unusual case.