ODCs

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1 OMIM reference -
1 associated gene
3 signs/symptoms

COMMON GENES: 1
COMMON SIGNS: 1

1 OMIM reference -
1 associated gene
6 signs/symptoms

Progressive symmetric erythrokeratodermia

Keratoderma hereditarium mutilans with ichthyosis

LOR

LOR

Citations in the biomedical literature:

Progressive symmetric erythrokeratodermia		Keratoderma hereditarium mutilans with ichthyosis
	Synonym(s): - Darier-Gottron disease - Progressiva symmetrica erythrokeratodermia - Progressive symmetric erythrokeratodermia, Gottron type		Synonym(s): - Camisa disease - Keratoderma - ichthyosiform dermatosis - elevated beta-glucuronidase - Loricrin keratoderma - Vohwinkel syndrome - ichthyosis

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

	Classification (ICD10): - Congenital malformations, deformations and chromosomal abnormalities -		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		Epidemiological data: (no data available)

	External references: 1 OMIM reference - No MeSH references		External references: 1 OMIM reference - No MeSH references


COMMON SIGNS	- Palmoplantar hyperkeratosis / keratoderma

Progressive symmetric erythrokeratodermia		Keratoderma hereditarium mutilans with ichthyosis
	Very frequent - Autosomal dominant inheritance - Erythema / erythematous lesions / erythroderma / polymorphous erythema		Very frequent - Ichthyosis / ichthyosiform dermatitis Occasional - Auto-aggressivity / auto-mutilation - Hypertonia / spasticity / rigidity / stiffness - Motor deficit / trouble - Nails anomalies