Orphanet: Peeling skin syndrome type B

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Peeling skin syndrome type B

Disease definition

Peeling skin syndrome (PSS) type B, also known as peeling skin disease (PSD), is a rare inflammatory form of ichthyosis (see this term) characterized by superficial patchy peeling of the entire skin with underlying erythroderma, pruritus, and atopy.


Classification level: Subtype of disorder
  • Synonym(s):
    • Generalized deciduous skin type B
    • Generalized peeling skin syndrome type B
    • Inflammatory peeling skin syndrome
    • PSS type B
  • Prevalence: <1 / 1 000 000
  • Inheritance: Autosomal recessive 
  • Age of onset: Infancy, Neonatal
  • ICD-10: Q80.8
  • OMIM: 270300
  • UMLS: -
  • MeSH: -
  • GARD: -
  • MedDRA: -

Detailed information


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