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Peeling skin syndrome

Synonym(s) Deciduous skin
Familial continuous skin peeling syndrome
Idiopathic deciduous skin
Keratosis exfoliativa congenita
Peeling skin disease
Prevalence Unknown
Inheritance Autosomal recessive
Age of onset Infancy
  • Q80.8
  • C0406357
MeSH -
MedDRA -


Disease definition

Peeling skin syndrome (PSS) refers to a group of rare autosomal recessive forms of ichthyosis (see this term) that is characterized clinically by superficial, asymptomatic, spontaneous peeling of the skin and histologically by a shedding of the outer layers of the epidermis. PSS presents with either an acral (acral PSS) or a generalized distribution (generalized PSS type A (non inflammatory) or B (inflammatory)) (see these terms). Some cases remain difficult to classify, suggesting that there could be additional subtypes of PSS.

Expert reviewer(s)

  • Dr Nathalie JONCA

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Detailed information

Practical genetics
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