Dermatopathia Pigmentosa Reticularis

Dermatopathia 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.^:856

Presentation

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

DPR is very similar to the related Naegeli-Franceschetti-Jadassohn syndrome. Both cause an affected person to lack fingerprints, have a lace-like pattern of hyperpigmentation and hyperkeratosis of the palms of the hands and soles of the feet. DPR is distinguished from NFJS by the duration of hyperpigmentation and lack of dental abnormalities.

Cause

DPR is caused by a mutation in the keratin 14 gene.

Treatment

See also

• List of cutaneous conditions caused by mutations in keratins