Peeling skin syndrome type A

Peeling skin syndrome type A (PSS type A), also known as acral peeling skin syndrome, is a rare inherited skin disorder characterized by painless, superficial peeling (exfoliation) of the outermost layer of the skin (epidermis), predominantly affecting the hands and feet (acral distribution). The condition is caused by mutations in the TGM5 gene, which encodes transglutaminase 5, an enzyme important for maintaining the structural integrity of the upper layers of the skin. The peeling is typically 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. Clinically, patients present with spontaneous or easily induced superficial skin peeling on the dorsal and palmar surfaces of the hands and the plantar surfaces of the feet. The peeling is generally painless and non-inflammatory, though mild redness (erythema) may occasionally be present beneath the peeled skin. Unlike peeling skin syndrome type B (the generalized form), type A is limited to acral sites and does not typically involve widespread body surfaces or systemic features such as atopy or immunological abnormalities. Blistering is generally absent, distinguishing this condition from epidermolysis bullosa and other blistering disorders. There is currently no curative treatment for peeling skin syndrome type A. Management is supportive and symptomatic, focusing on skin protection and moisturization. Emollients and keratolytic agents may help reduce peeling and improve skin appearance. Patients are advised to minimize exposure to triggers such as excessive moisture, heat, and friction. The condition is generally benign and does not affect overall health or life expectancy, though it can cause cosmetic concern and mild functional discomfort.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Common questions about Peeling skin syndrome type A