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Dyskeratosis Congenita with Pigmentation, Dystrophia Unguium, and Leukoplakia OrisA Follow-Up Report of Two Brothers
JOHN GARB, M.D.
AMA Arch Derm. 1958;77(6):704-712.
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A previous publication1 dealt with two Jewish-American brothers, aged 25 and 21, Cases 1 and 2, who had in common the main features of the very rare syndrome dyskeratosis congenita with pigmentation, dystrophia unguium, and leukoplakia oris. This paper completes their interesting and tragic life histories.
Review of Case 1
When the patient was 5 years of age, blisters appeared at the sides of the tongue. These healed after medication but recurred yearly. Sores later developed on the inside of the mouth and gums. Attacks of dysphagia occurred when the patient was 8 years old, due to narrowing of the esophageal orifice. At the age of 11 the teeth began to soften, to crumble, and gradually to decay. The trunk and extremities revealed pigmentary changes. At the age of 12 the nails of the hands became dystrophic, shriveled, and shrunken. The skin was dry except for the palms
. . . [Full Text PDF of this Article]
Author Affiliations
New York
From the Department of Dermatology and Syphilology of the New York University Post-Graduate Medical School (Dr. Marion B. Sulzberger, Chairman) and Skin and Cancer Unit of New York University Hospital.
Footnotes
Submitted for publication Nov. 26, 1957.
Read before the 11th International Congress of Dermatology, Stockholm, Aug. 1, 1957.
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