Abstract

We present an 81-year-old woman with rheumatoid arthritis on methotrexate, and a left upper eyelid nodule of at least 6-months duration.  She was initially treated as a potential chalazion and lack of response to therapy resulted in an incisional biopsy.  Histopathology demonstrated a dense lymphoplasmacytic infiltrate with pleomorphism and high mitotic activity with overlying ulceration that was teeming with hyphal elements.  Immunohistochemical stains demonstrated a mixed CD3 and CD20 lymphocytic infiltrate with abundant plasma cells that were positive for EBER. Lambda restriction also was noted consistent with EBV lymphoproliferative disorder.  The clinical presentation of a solitary nodule in the setting of methotrexate use raised the possibility of EBV mucocutaneous ulcer. Tissue culture grew Purpureocilium lilacinum within 3 days.  Purpureocilium lilacinum is an emerging, rare, opportunistic pathogenic fungus that has been associated with ocular, as well as cutaneous and systemic disease, primarily in immunocompromised patients. Ocular infections present with keratitis or endophthalmitis, and cutaneous infections have varied morphologies including single or grouped red macules, pseudo-verrucous papules, vesicles, non-ulcerated and ulcerated nodules.  Although limited by case reports, this fungal infection has demonstrated resistance to antifungals, making it more difficult to treat. This case is exceptional for the concomitant presence of EBV positivity and atypia characteristic of lymphoproliferative disorder, and fungi, calling into question whether both occurred simultaneously or if one – infection or malignancy – potentially stimulated the development of the other. The patient’s underlying immunosuppression likely contributed to development of both, and treatment included elimination of her immunosuppression.