Ocular surface squamous neoplasia in Papillon-Lefevre syndrome.

PURPOSE: To report the occurrence of ocular surface squamous neoplasia (OSSN) in a patient with Papillon-Lefevre syndrome.

DESIGN

Interventional case report.

METHODS

A 14-year-old boy presented with a progressively growing limbal lesion in the right eye for 2 months. He had associated erythematous scaly lesions on the elbows, knees, and feet with periodontosis suggestive of Papillon-Lefevre syndrome. An excision biopsy of the limbal tumor and a skin biopsy was performed.

RESULTS

The limbal tumor showed features of carcinoma in situ with clear margins. The skin biopsy showed epidermal hyperplasia and hyperkeratosis with perivascular infiltrates, consistent with Papillon-Lefevre syndrome.

CONCLUSIONS

Ocular surface squamous neoplasia may occur in patients with Papillon-Lefevre syndrome, an association that to the best of our knowledge has not been previously reported.