Dermatopathia pigmentosa reticularis

Dermatopathia pigmentosa reticularis
Dermatopathia pigmentosa reticularis has an autosomal dominant pattern of inheritance
Classification and external resources
Specialty	medical genetics
ICD-10	Q82.4
OMIM	125595
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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 dominantcongenital disorder that is a form of ectodermal dysplasia. Dermatopathia pigmentosa reticularis is composed of the triad of generalized reticulate hyperpigmentation, noncicatricial alopecia, and onychodystrophy.

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

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

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