Diffuse keratoses syndrome    368

General: Ophthalmic complications and manifestations following a group of dermatologic conditions for which the etiology is unknown, including scleroderma, CRST syndrome, Rendu-Osler- Weber syndrome, progressive systemic sclerosis with calcinosis, Spanlang-Tappeiner syndrome, and Savin syndrome (see Rendu-Osler-Weber Syndrome; Progressive Systemic Sclerosis; Spanlang-Tappeiner Syndrome; Savin Syndrome; CRST Syndrome; Scleroderma).

Ocular: Dermatologic and cicatricial skin changes; hydrophthalmos; corneal changes with nodular thickening in the stroma, which worsen in fall and improve during spring (particularly Savin syndrome); retinal phlebitis.

Clinical: Ichthyosis (usually congenital); microcephalus (CRST); facial dermatoses; deafness; urticarial (allergic) manifestations; subcutaneous calcinous plaques, mainly on hands; sclerodactyly.

Geeraets WJ. Ocular Syndromes, 3rd ed. Philadelphia: Lea & Febiger, 1976

Kleine-Natrop HE, Haustein UF. 'Benign mucus membrane pemphigoid' with rapid loss of sight and generalized scarring skin change. Hautarzt 1968; 19:6.

Savin LH. Corneal dystrophy associated with congenital ichthyosis and allergic manifestations in male members of a family. Br J Ophthalmol 1956; 40:82.

Thibierge G, Weissenbach RJ. Concretions Calcaires Souscutanees et Sclerodermie. Ann Dermatol Syph 1911; 2:129.