Inflammatory peeling skin syndrome studied with electron microscopy

Peeling skin syndrome is a rare autosomal recessive dermatosis characterized by lifelong continual skin shedding with underlying erythema. We present a 10-year-old boy with this disease. Easy peeling of the skin associated with keratotic areas was observed. Microscopic findings included psoriasiformis dermatitis. Cleavage was observed between corneocytes as well as between granular-corneum cells and within the granular cell layer. Electron microscopic findings included intra- and intercellular cleavage of corneocytes and intracellular separation of granular cells. The molecular mechanisms of peeling skin syndrome are discussed.