Acral peeling skin syndrome
Pronunciation: AY-kral PEEL-ing skin SIN-drohm
A rare autosomal recessive genodermatosis characterised by painless, superficial peeling of the skin limited to the hands and feet, often worsening with moisture or friction.
Full Definition
Acral peeling skin syndrome (APSS) is a hereditary disorder caused by mutations in the TGM5 gene encoding transglutaminase 5, disrupting corneocyte cohesion in the acral stratum granulosum. It presents in infancy or early childhood with blistering and painless superficial peeling confined to the dorsal and plantar surfaces of the hands and feet. The condition is non-inflammatory and does not involve mucous membranes, distinguishing it from epidermolysis bullosa and other mechanobullous disorders. Editors must be careful not to conflate it with generalised peeling skin syndrome, which involves the entire body surface. Clinical reports typically describe exacerbation by warmth, sweating, or water exposure.
Usage
Usage note: Distinguish carefully from generalised peeling skin syndrome; 'acral' specifies restriction to hands and feet and must not be dropped or replaced.
In Context
- "The case report documented a 3-year-old with recurrent peeling of the dorsal hands since infancy, confirmed genetically as acral peeling skin syndrome." — Pediatric dermatology case report
- "Editors should not substitute 'acral peeling skin syndrome' with the broader term 'peeling skin syndrome' without verifying the distribution pattern described." — Editorial style note for clinical manuscript