Continual skin peeling syndrome. An electron microscopic study

We encountered a patient with continual skin peeling syndrome, a rare disorder in which generalized, noninflammatory exfoliation of the stratum corneum occurs. Although scaling occurred spontaneously in our patient, he was also able to manually peel sheets of skin without bleeding or pain. Histologically, there was separation of corneocytes above the granular cell layer. Ultrastructural examination revealed an unusual type of intracellular cleavage, in which the plasma membrane of the "peeling" cell remained firmly adherent to the underlying cell while the upper part of the cell exfoliated. Unique intercellular electron-dense globular deposits were localized to the stratum corneum.