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April 1955

Dyskeratosis Congenita with Pigmentation, Dystrophia Unguium, and Leucokeratosis Oris: Review of the Known Cases Reported to Date and Discussion of the Disease from Various Aspects

Author Affiliations


From the Department of Dermatology and Syphilology, Western Reserve Medical School, University Hospitals, and the Cleveland City Hospital.

AMA Arch Derm. 1955;71(4):451-456. doi:10.1001/archderm.1955.01540280027005

In October, 1926, a unique case was presented before the Annual Meeting of the Cleveland Dermatological Society by Drs. Cole, Rauschkolb, and Toomey.1 This 20-year-old man of Jewish extraction stated that as far back as he could remember he had had a peculiar condition of his nails and an unusual pigmentation around his neck. He complained that his hands and feet perspired very freely and he often had large blisters over his heels. His father, mother, and three sisters were living and well.

At that time, the entire tongue had a thickened, whitish appearance, with a large ulcer in its center. Later, there was a large leucoplakic spot on the hard palate. The nails were dystrophic in type, and some of them were almost entirely absent. The skin of the neck down to the clavicle showed fine dark reddish-brown papules, arranged along the somites of the skin.