Dermatopathia pigmentosa reticularis
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OMIM | 125595 |
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Dermatopathia pigmentosa reticularis (DPR) is a form of a rare sex-linked ectodermal dysplasia congenital disorder that afflicts females. Symptoms include lack of sweat glands, thin hair, brittle nails, mottled skin, and lack of fingerprints. The disorder is so rare that only the Maynard family line carries the disorder.
DPR is comparable to Naegeli syndrome, both of which is caused by a specific defect in the keratin 14 protein.