Orphanet: Peeling skin syndrome

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Peeling skin syndrome

Disease definition

A group of rare autosomal recessive forms of ichthyosis clinically characterized 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 for generalized PSS type A (noninflammatory) or B (inflammatory).


Classification level: Group of disorders
  • 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: -
  • ICD-11: EC20.1
  • OMIM: -
  • UMLS: C1849193
  • MeSH: -
  • GARD: 7347
  • MedDRA: -

Detailed information

ERN : produced/endorsed by ERN(s)
FSMR : produced/endorsed by FSMR(s)
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