peeling skin syndrome (keratosis exfoliativa congenita)

Introduction

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

More general terms

• genetic disease of the skin (genodermatosis)
• syndrome

More specific terms

• peeling skin syndrome acral type

Database

• OMIM: https://mirror.omim.org/entry/270300