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Progressive symmetric erythrokeratodermia
1 OMIM reference -
1 associated gene
8 connected diseases
3 signs/symptoms
Disease Type of connection
Keratoderma hereditarium mutilans with ichthyosis
Annular epidermolytic ichthyosis
Epidermolytic ichthyosis
Epidermolytic palmoplantar keratoderma
Exfoliative ichthyosis
Ichthyosis hystrix of Curth-Macklin
Keratosis palmoplantaris striata
Pulverulent cataract
Synonym(s):
- Darier-Gottron disease
- Progressiva symmetrica erythrokeratodermia
- Progressive symmetric erythrokeratodermia, Gottron type

Classification (Orphanet):
- Rare genetic disease
- Rare skin disease

Classification (ICD10):
- Congenital malformations, deformations and chromosomal abnormalities -

Epidemiological data:
Class of prevalence: unknown
Average age onset: neonatal/infancy
Average age of death: normal
Type of inheritance: autosomal dominant
External references:
1 OMIM reference -
No MeSH references

Gene symbol UniProt reference OMIM reference
LOR P23490152445
Very frequent
- Autosomal dominant inheritance
- Erythema / erythematous lesions / erythroderma / polymorphous erythema
- Palmoplantar hyperkeratosis / keratoderma