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Disorder that results in the combination of teeth and nail anomalies with hypohidrosis.


Extremely rare, autosomal dominant inherited disorder described in two families.


Hypoplastic, hypocalcified enamel of teeth; onycholysis and subungual hyperkeratosis; hypohidrosis.


Obtain history and document loose teeth. Careful direct laryngoscopy is required to prevent damage to fragile teeth. Hypohidrosis may result in temperature disturbances, and atropine should be used carefully.

Witkop CJ Jr, Brearley LJ, Gentry WC Jr: Hypoplastic enamel, onycholysis, and hypohidrosis inherited as an autosomal dominant trait. A review of ectodermal dysplasia syndromes. Oral Surg Oral Med Oral Pathol 39:71, 1975.  [PubMed: 122795]

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