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peeling skin syndrome (keratosis exfoliativa congenita)

Two main subtypes, noninflammatory type A & inflammatory type B, have been suggested. However, there are additional subtypes. Pathology: - ultrastructural & histological examination shows a level of blistering high in the epidermis at the stratum granulosum- stratum corneum junction Genetics: - autosomal recessive Clinical manifestations: - continuous shedding of the outer layers of the epidermis from birth & throughout life - in some cases of PSS, skin peeling is accompanied by erythema, vesicular lesions, or, in rare cases, other ectodermal f eatures, like fragile hair & nail abnormalities

Specific

peeling skin syndrome acral type

General

genetic disease of the skin (genodermatosis) syndrome

Database Correlations

OMIM 270300

References

  1. OMIM :accession 270300
  2. UniProt :accession O43548