Acral peeling skin syndrome

Acral peeling skin syndrome (APSS) is a rare inherited skin disorder characterized by painless, superficial peeling (exfoliation) of the outermost layer of the skin (stratum corneum), predominantly affecting the hands and feet (acral areas). It belongs to the broader group of peeling skin syndromes and is classified under the ichthyoses. The condition is also known as acral peeling skin disease or peeling skin syndrome type 1 (acral type). It is caused by mutations in the TGM5 gene, which encodes transglutaminase 5, an enzyme important for maintaining the structural integrity of the epidermis. Less commonly, mutations in the CSTA gene (encoding cystatin A) have also been identified in some patients. The peeling of skin in APSS is typically non-inflammatory and painless, though it can be worsened by heat, humidity, moisture exposure, and mechanical friction. Onset is usually from birth or early childhood, and the condition tends to persist throughout life. The peeling is superficial and generally does not cause scarring, though it can be cosmetically distressing. Some patients may experience mild redness (erythema) or itching (pruritus) in affected areas. Blistering may occasionally occur. The condition is limited to the skin and does not typically affect other organ systems. There is currently no curative treatment for acral peeling skin syndrome. Management is supportive and symptomatic, focusing on skin protection and moisturization. Emollients and barrier creams are commonly used to reduce skin dryness and minimize peeling. Patients are advised to avoid triggers such as excessive heat, moisture, and mechanical trauma to the skin. Keratolytic agents may be used cautiously in some cases. The prognosis is generally good, as the condition is benign and does not affect life expectancy or overall health, though it can significantly impact quality of life due to its chronic and visible nature.

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