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Generalized peeling skin syndrome

Disease definition

Generalized peeling skin syndrome (PSS) is a form of PSS (see this term) presenting with a generalized distribution. It comprises two sub-types: the non-inflammatory (PSS type A) and the inflammatory (PSS type B) form (see these terms). PSS type A is characterized by generalized white scaling with superficial peeling of the skin, while PSS type B is characterized by superficial patchy peeling of the entire skin with underlying erythroderma, associated with pruritus, and atopy.

ORPHA:263543

Classification level: Disorder
  • Synonym(s):
    • Generalized PSS
    • Generalized deciduous skin
  • Prevalence: <1 / 1 000 000
  • Inheritance: Autosomal recessive 
  • Age of onset: Infancy, Neonatal
  • ICD-10: Q80.8
  • OMIM: 270300  616265
  • UMLS: -
  • MeSH: -
  • GARD: 12862
  • MedDRA: -

Detailed information

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