Cutis marmorata syndrome (marble skin syndrome)  338

General: Prominent in children; etiology unknown; possibly a mild form of livedo reticularis; in severe cases, vessel dilation and skin changes may be permanent; occurs with exposure to cold and subsides in a warm environment; extremities and pectoral region most frequently affected.

Ocular: Congenital glaucoma; corneal and scleral thinning; staphyloma; sclerotic appearance to trabecular meshwork; corneal edema; cataracts; optic nerve atrophy; heterochromia iridium; iris anterior layer dysplasia; intraoperative suprachoroidal hemorrhage (rare).

Clinical: Bluish-red mottling of skin; spasmodic narrowing of arterioles with dilation of vessels; ulceration and scaling of skin; congenital hypothyroidism.

Champion RH. Livedo reticularis: a review. Br J Dermatol 1965; 77:167-l79.

Kremer I, et al. Intraoperative suprachoroidal hemorrhage in congenital glaucoma associated with cutis marmorata telangiectatica congenita. Arch Ophthalmol 1991; 109:1199-l200.

Magalini SI, Scrascia E. Dictionary of Medical Syndromes. 2nd ed. Philadelphia: JB Lippincott, 1981.

Mayatepek E, et al. Congenital glaucoma in cutis marmorata telangiectatica congenita. Ophthalmologica 191-l93.

Pehr K, Moroz B. Cutis marmorata telangiectatica congenita: long-term follow-up, review of the literature, and report of a case in conjunction with congenital hypothyroidism. Pediatr Dermatol 1993; 10:6-l1.

Sato SE, et al. Congenital glaucoma associated with cutis marmorata telangiectatica congenita: two case reports. J Pediatr Ophthalmol Strabismus 1988; 25: 13-l7.