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Peeling skin syndrome

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.

ORPHA:817

  • Synonym(s):
    • Deciduous skin
    • Familial continuous skin peeling syndrome
    • Idiopathic deciduous skin
    • Keratosis exfoliativa congenita
    • PSS
    • Peeling skin disease
  • Prevalence: Unknown
  • Inheritance: Autosomal recessive 
  • Age of onset: Infancy, Neonatal
  • ICD-10: Q80.8
  • OMIM: -
  • UMLS: C0406357
  • MeSH: -
  • GARD: 7347
  • MedDRA: -

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