Peeling Skin Syndrome 5 (PSS5) is a genodermatosis characterized by superficial, painless peeling of the skin. Affected areas include the dorsal and palmar surfaces of the hands and feet, as well as the fore-arms and legs. Peeling is usually seen to be exacerbated by external factors such as heat, humidity or friction. Other symptoms include underlying erythema and diffuse yellowish hyperkeratotic palmoplantar plaques. Skin biopsies may reveal acanthosis, hyperkeratosis, disadhesion of keratinocytes in basal and suprabasal layers of epidermis and widening of intercellular spaces.
The condition has an onset at around 3 to 6 months of age. While PSS is an uncommon disorder, with less than 100 cases reported worldwide, PSS5 is a rare subset of PSS, with only about 3 reported incidences. Diagnosis of PSS5 is based on clinical features and genetic analysis of the SERPINB8 gene. Treatment is limited to the use of emollient ointments to provide relief.