Abstract

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.