Posters

Bilateral spontaneous corneal perforations in a patient with follicular dendritic cell sarcoma and distant history of paraneoplastic pemphigus

Poster Details

First Author: O.Klavdianou GREECE

Co Author(s):    N. Venkateswaran   G. Kondylis   I. Kosmidis   S. Palioura   .         

Abstract Details

Purpose:

To report a case of bilateral spontaneous corneal perforations in a patient with follicular dendritic cell sarcoma and distant history of paraneoplastic pemphigus.

Setting:

Athens Vision Eye Institute, Cornea Service, Athens, Greece

Methods:

A 73-year-old woman presented with bilateral decrease in vision. The patient has a history of follicular dendritic cell sarcoma, which was originally diagnosed following a mucosal eruption, consistent with paraneoplastic pemphigus and managed with the CHOP regimen. Two months prior the regimen changed to gemcitabine and prednisone. Slit lamp examination revealed bilateral corneal perforations with iris plugging measuring 2 x 2 mm OD and 1 x 1 mm OS, with mild forniceal foreshortening bilaterally. Multi-layered amniotic membrane transplantation was performed OD and cyanoacrylate glue was applied OS. The patient received methylprednisolone and cyclophosphamide for presumed reactivation of her paraneoplastic pemphigus.

Results:

Both lesions improved significantly. Three cases have been described with follicular dendritic cell lymphoma-associated paraneoplastic pemphigus, of which two had conjunctival hyperemia during the acute phase of the disease. Ocular involvement in paraneoplastic pemphigus has been described in eleven patients. In ten patients ocular manifestations occured at the acute phase, while in two patients ocular involvement was developed in the chronic phase of paraneoplastic pemphigus. Corneal perforations were developed in two patients at the acute phase of the disease. Overall, our patient is the first case of bilateral corneal perforations described in the chronic phase of paraneoplastic pemphigus.

Conclusions:

Bilateral spontaneous corneal perforations are a hallmark of systemic disease. In this case, we believe that the switch in the patient’s chemotherapy regimen led to reactivation of her latent paraneoplastic pemphigus, with severe ocular sequelae. Review of the literature indicates that paraneoplastic pemphigus can rarely be associated with spontaneous corneal perforations and should be managed with aggressive topical and systemic therapy.

Financial Disclosure:

None