Dermatopathia pigmentosa reticularis, abbreviated DPR, is a rare inherited disorder passed on in an autosomal dominant pattern and present from birth. It is classed as a form of ectodermal dysplasia and has also been identified as a keratin disorder. The condition is defined by a triad of signs: generalized reticulate hyperpigmentation of the skin, non-scarring hair loss, and malformed nails. It chiefly affects the skin, hair and nails and is not life threatening. Very few cases are on record: as of 1992 only ten had been described in the medical literature, and five further families studied in 2006 with modern genetic analysis added to that short list of confirmed cases.
Facts
Classification
ICD-10 ChapterXVII: Congenital Malformations, Deformations and Chromosomal Abnormalities 2 Communicable or Non-communicable Transmission Route Causative Agent
Causative Agent (category) Prevalence Band Sources
1. Dermatopathia pigmentosa reticularis (Wikipedia)
Lead
Dermatopathia pigmentosa reticularis (DPR) is a rare, autosomal dominant congenital disorder that is a form of ectodermal dysplasia.
Lead section
rare, autosomal dominant congenital disorder that is a form of ectodermal dysplasia
Lead section [body-system]
largely affects the skin, hair, and nails
View the Source2. Orphadata (Orphanet): Orphadata cross-references for ORPHA:86920 Dermatopathia pigmentosa reticularis
Orphadata (Orphanet)ICD-10 cross-referenceQuote, ICD-10 cross-reference
"Reference": "Q82.4", "Source": "ICD-10"
View the Source 3. Orphadata (Orphanet): Orphadata epidemiology for ORPHA:86920 Dermatopathia pigmentosa reticularis
Orphadata (Orphanet)Prevalence, WorldwideQuote, Prevalence, Worldwide
"PrevalenceClass": "\u003c1 / 1 000 000", "PrevalenceGeographic": "Worldwide"
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