Dermatopathia pigmentosa reticularis





Dermatopathia pigmentosa reticularis (DPR), also known as dermatopathia pigmentosa reticularis hyperkeratotica et mutilans, dermatopathia pigmentosa reticularis hypohidotica et atrophica and dermatopathic pigmentosa reticularis, is a rare, autosomal dominant congenital disorder that is a form of ectodermal dysplasia. Dermatopathia pigmentosa reticularis is composed of the triad of generalized reticulate hyperpigmentation, noncicatricial alopecia, and onychodystrophy.

Presentation



Symptoms include lack of sweat glands, thin hair, brittle nails, mottled skin, and lack of fingerprints.

Cause



DPR is comparable to Naegeli syndrome, both of which are caused by a specific defect in the keratin 14 protein.

See also



  • Naegeliâ€"Franceschettiâ€"Jadassohn syndrome
  • List of cutaneous conditionsli>
  • List of cutaneous conditions caused by mutations in keratins

References



External links



  • Scientists Unravel Mystery of People with No Fingerprints
  • Medical Mystery: No Fingerprints




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