Dermatopathia pigmentosa reticularis
Dermatopathia pigmentosa reticularis |
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Classification and external resources |
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ICD-10 |
Q82.4 |
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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,[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]
![](../I/m/Autosomal_dominant_-_en.svg.png)
Dermatopathia pigmentosa reticularis has an autosomal dominant pattern of
inheritance.
See also
References
External links
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| Genodermatosis | Congenital ichthyosis/ erythrokeratodermia | |
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| EB and related |
- EBS
- EBS-K
- EBS-WC
- EBS-DM
- EBS-OG
- EBS-MD
- EBS-MP
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| Ectodermal dysplasia | |
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| Elastic/Connective | |
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| Hyperkeratosis/ keratinopathy | PPK |
- diffuse: Diffuse epidermolytic palmoplantar keratoderma
- Diffuse nonepidermolytic palmoplantar keratoderma
- Palmoplantar keratoderma of Sybert
- Mal de Meleda
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| Other | |
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| Other |
see also Template:Congenital malformations and deformations of skin appendages, Template:Phakomatoses, Template:Pigmentation disorders, Template:DNA replication and repair-deficiency disorder
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| Developmental anomalies | Midline | |
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| Nevus | |
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| Other/ungrouped | |
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| Description |
- Anatomy
- Physiology
- Development
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| Disease |
- Infections
- Vesiculobullous
- Dermatitis and eczema
- Papulosquamous
- Urticaria and erythema
- Radiation-related
- Pigmentation
- Mucinoses
- Keratosis, ulcer, atrophy, and necrobiosis
- Vasculitis
- Fat
- Neutrophilic and eosinophilic
- Congenital
- Neoplasms and cancer
- nevi and melanomas
- epidermis
- dermis
- Symptoms and signs
- Terminology
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| Treatment |
- Procedures
- Drugs
- antibiotics
- disinfectants
- emollients and protectives
- itch
- psoriasis
- other
- Wound and ulcer
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Genetic disorder, extracellular: scleroprotein disease (excluding laminin and keratin) |
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| Collagen disease | COL1: | |
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| COL2: | |
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| COL3: | |
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| COL4: | |
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| COL5: | |
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| COL6: | |
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| COL7: | |
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| COL8: | |
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| COL9: | |
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| COL10: | |
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| COL11: | |
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| COL17: | |
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| Laminin | |
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| Other | |
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| see also fibrous proteins
Index of cells |
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| Description |
- Structure
- Organelles
- peroxisome
- cytoskeleton
- centrosome
- epithelia
- cilia
- mitochondria
- Membranes
- Membrane transport
- ion channels
- vesicular transport
- solute carrier
- ABC transporters
- ATPase
- oxidoreduction-driven
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| Disease |
- Structural
- peroxisome
- cytoskeleton
- cilia
- mitochondria
- nucleus
- scleroprotein
- Membrane
- channelopathy
- solute carrier
- ATPase
- ABC transporters
- other
- extracellular ligands
- cell surface receptors
- intracellular signalling
- Vesicular transport
- Pore-forming toxins
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