Peeling skin syndrome type B

Peeling skin syndrome type B (PSS type B), 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). Unlike peeling skin syndrome type A, which involves generalized peeling across the body, type B is localized to the extremities. 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. Loss of function of this enzyme leads to impaired cohesion within the stratum corneum, resulting in spontaneous or mechanically induced skin peeling. The peeling is typically painless and non-inflammatory, though it may worsen with heat, humidity, moisture exposure, or mechanical friction. Affected individuals may notice continuous or episodic shedding of thin sheets of skin from the palms, soles, and dorsal surfaces of the hands and feet. The underlying skin may appear mildly reddened but generally heals without scarring. Onset is usually from birth or early childhood, and the condition persists throughout life, though severity may fluctuate. The disorder primarily affects the integumentary system (skin) and does not typically involve other organ systems. There is currently no cure for peeling skin syndrome type B. Treatment is symptomatic and supportive, focusing on skin protection and moisturization. Regular application of emollients and barrier creams can help reduce peeling and protect exposed skin. Patients are advised to minimize friction, excessive moisture, and heat exposure. Keratolytic agents are generally avoided as they may worsen peeling. The condition is benign and does not affect life expectancy, though it can cause cosmetic concern and mild functional discomfort.

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