Rare disease: Paraneoplastic pemphigus: an uncommon cause of chronic cicatrising conjunctivitis

摘要

We report a case of paraneoplastic pemphigus (PNP) as an uncommon but severe cause of cicatrising conjunctivitis. Initially diagnosed as drug eruptions, the patient’s condition did not improve despite cessation of chemotherapy. Immunohistological confirmation of PNP has led to the use of combined oral prednisolone and intravenous immunoglobulin. Her ocular conditions stabilised with complete recovery of vision. PNP is a rare disease that can present with ocular involvement. Ophthalmologists should play an active role in monitoring and treatment of ocular surface complications such as symblepharon formation, severe dry eye and epithelial breakdown. Vigorous and prompt treatment is the key to successful prevention of irreversible and blinding complications. The atypical feature in this case is the presence of eosinophilic infiltration on histology that is a feature of allergic aetiologies rather than classical PNP.