Von sallmann-paton-witkop syndrome (hereditary benign intraepithelial dyskeratosis; witkop-von sallmann syndrome; hbid syndrome) 1

General: Autosomal dominant; conjunctival and. oral lesions; found in whites.

Ocular: Foamy gelatinous plaques located in a typical horseshoe fashion at 3 and 9 o'clock positions of the perilimbal area; superficial hyperemia of bulbar conjunctiva; corneal vascularization and consequent visual decrease.

Clinical: Thickening of oral mucosa with whitish plaques and folds of a spongy character (asymptomatic), with slow progression from birth into second decade of life.

McLean IW; et al. Hereditary benign intraepithelial dyskeratosis. Ophthalmology 1981; 88:164-l68.

von Sallmann L, Paton D. Hereditary benign intraepithelial dyskeratosis: I. Ocular manifestations. Arch Ophthalmol 1960; 63:421.

Witkop CJ Jr, et al. Hereditary benign intraepithelial dyskeratosis: II. Oral manifestations and hereditary transmission. Arch Pathol 1960; 70:696.