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Pachyonychia Congenita With Cardiac Involvement

S. Premalatha, MD, DD; A. S. Thambiah, FRCP, FAMS
Arch Dermatol. 1977;113(5):687. doi:10.1001/archderm.1977.01640050147035.
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To the Editor.—  A case of pachyonychia congenita with cardiac involvement has been recorded and reported. A 9-year-old girl had all the characteristic features of pachyonychia congenita. In addition, there was clinical and roentgenographic evidence of cardiomegaly, with abnormal ECG and angiographic studies. She also had a pigmented nevus in the left iris, spinabifida occulta L5, rudimentary 12th rib, sparse kinky hair over the scalp, and carious teeth.

Report of a case.—  A 9-year-old girl born to consanguinous parents was brought to the Dermatology Department on Aug 18, 1974.When her history was taken, it was noted that approximately three months after birth blisters in frictional areas started to develop. At the age of 7 months, horny follicular lesions mostly over the extremities developed. Nail dystrophy in the form of thickening and lifting up of the nail plates started at the age of 1 year. When the child started walking,


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