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April 1985

Picture of the Month

Author Affiliations

Contributed from the Department of Genetics and Birth Defects, Kennedy Memorial Hospital, Brighton, Mass.

Am J Dis Child. 1985;139(4):425-426. doi:10.1001/archpedi.1985.02140060107043

Denouement and Discussion 

Syndromes Associated With Nail Dysplasia 

Diagnoses  Hydrotic Ectodermal Dysplasia.—The severity of the nail dysplasia varies and involves both fingernails and toenails. Facial appearance is normal, but scalp hair may be absent or sparse. In contrast with the hypo-hydrotic type of ectodermal dysplasia, the sweat glands and teeth are normal. It is inherited in an autosomal dominant manner.Turner's Syndrome.—Toenails are more frequently dysplastic than are fingernails. Lymphedema is present over the dorsum of the hands and feet. Major findings include a short, webbed neck, ovarian dysgenesis, lack of secondary sexual characteristics, congenital heart defects, short stature, and usually only one X chromosome.Hereditary Osteo-onychodysplasia (Nail-Patella Syndrome).—The dysplastic nails may be small and narrow and split easily. Patellas are usually absent but may be hypoplastic. Other skeletal abnormalities include iliac horns, elbow anomalies, and scoliosis. Glomerulonephritis and nephrosis

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