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


METHODS
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.
Murthy
Ramesh
R
Ocular Oncology Service, LV Prasad Eye Institute, Hyderabad, India.
Honavar
Santosh G
SG
Vemuganti
Geeta K
GK
Burman
Sanghamitra
S
Naik
Milind
M
Parathasaradhi
A
A
D000293:Adolescent; D001706:Biopsy; D003316:Corneal Diseases / Q000473:pathology*; D005134:Eye Neoplasms / Q000473:pathology*; D006801:Humans; D016850:Limbus Corneae / Q000473:pathology*; D008297:Male; D018307:Neoplasms, Squamous Cell / Q000473:pathology*; D010214:Papillon-Lefevre Disease / Q000473:pathology*; D012867:Skin / Q000473:pathology