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Transgrediens et progrediens palmoplantar keratoderma
1 OMIM reference -
1 associated gene
4 connected diseases
10 signs/symptoms
Disease Type of connection
Autosomal dominant nonsyndromic sensorineural deafness type DFNA
Autosomal recessive nonsyndromic sensorineural deafness type DFNB
Erythrokeratodermia variabilis
Neuropathy with hearing impairment
Synonym(s):
- Greither disease
- Keratosis extremitatum hereditaria progrediens
- Keratosis palmoplantaris transgrediens et progrediens
- Progressive diffuse PPK
- Progressive diffuse palmoplantar keratoderma
- Transgrediens et progrediens PPK
Classification (Orphanet):
- Rare genetic disease
- Rare skin disease
Classification (ICD10):
- Congenital malformations, deformations and chromosomal abnormalities -
Epidemiological data:
(no data available)
External references:
1 OMIM reference -
No MeSH references
Gene symbol UniProt reference OMIM reference
GJB3 O75712603324
Very frequent
- Autosomal dominant inheritance
- Dry / squaly skin / exfoliation
- Palmoplantar hyperkeratosis / keratoderma

Frequent
- Erythema / erythematous lesions / erythroderma / polymorphous erythema
- Hyperhidrosis / increased sweating
- Thin / hypoplastic / hyperconvex fingernails

Occasional
- Alopecia
- Corneal dystrophy
- Hyperkeratosis / ainhum / hyperkeratotic skin fissures
- Restricted joint mobility / joint stiffness / ankylosis