Dermatopathia pigmentosa reticularis
| Dermatopathia pigmentosa reticularis | |
|---|---|
| Classification and external resources | |
| Specialty | Lua error in Module:Wikidata at line 446: attempt to index field 'wikibase' (a nil value). |
| ICD-10 | Q82.4 |
| OMIM | 125595 |
| Patient UK | 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,[1]:511 is a rare, autosomal dominant[2] 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.[3]:856
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.[4]
See also
- Naegeli–Franceschetti–Jadassohn syndrome
- List of cutaneous conditions
- List of cutaneous conditions caused by mutations in keratins
References
- ↑ Freedberg, et al. (2003). Fitzpatrick's Dermatology in General Medicine. (6th ed.). McGraw-Hill. ISBN 0-07-138076-0.
- ↑ Lua error in package.lua at line 80: module 'strict' not found.
- ↑ James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology. (10th ed.). Saunders. ISBN 0-7216-2921-0.
- ↑ Lua error in package.lua at line 80: module 'strict' not found.
External links
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