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December 1962

An Acromegaloid, Cutis Verticis Gyrata, Corneal Leukoma Syndrome: A New Medical Entity

Author Affiliations

New Orleans
Department of Ophthalmology, Tulane University Medical School (Dr. Rosenthal).
Department of Anatomy, Tulane University Medical School (Dr. Kloepfer).

Arch Ophthalmol. 1962;68(6):722-726. doi:10.1001/archopht.1962.00960030726004

Introduction  Although the syndrome of cutis verticis gyrata associated with acromegaly, is well known in dermatological circles in this country and abroad, an exhaustive search of the literature has failed to reveal a case with the following additional findings: (1) developmental corneal leukomata; (2) longitudinal splitting of dermal ridges in the palm; (3) presence of a 100% penetrant autosomal dominant gene; (4) enlargement of the lateral half of the supraorbital arch of the frontal bone into a horn-like but skin-covered projection.Cutis verticis gyrata is a type of dermatolysis wherein the scalp is enlarged, causing gyrus-like formations.Out of the kindred of over 65 individuals, we have been able to examine 32. Of these, 13 were affected and 19 were unaffected.

General Description of the Syndrome  This syndrome includes 3 physical components: corneal leukomata, appearance of acromegaly, and cutis verticis gyrata. Corneal leukomata of a developmental character, beginning in childhood

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