Peeling Skin Syndrome [Supplementary Concept]
A rare, hereditary, autosomal recessive genodermatosis with onset from birth to adulthood. Clinically, it is characterized by spontaneous superficial peeling of the skin with or without PRURITIS, which sometimes is accompanied by ERYTHEMA or vesiculation. The skin involvement is usually general, but in some patients the scalp, face, palms, and soles may be unaffected. Seasonal changes have been reported. Histologically, it is characterized by separation of the EPIDERMIS between the statum corneum and the stratum granulosum. Mutations in the CDSN gene have been identified. OMIM: 270300
Date introduced: November 5, 2012
MeSH Unique ID: C564818
Heading Mapped to:
Entry Terms:
- Peeling skin syndrome 1
- Skin Peeling, Familial Continuous Generalized
- Keratolysis Exfoliativa Congenita
- Deciduous Skin