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March 1970

Porokeratosis—A Mutant Clonal Keratosis of the EpidermisI. Histogenesis

Author Affiliations

New Orleans

From the Department of Pathology, Tulane University School of Medicine, New Orleans.

Arch Dermatol. 1970;101(3):340-347. doi:10.1001/archderm.1970.04000030084014

Porokeratosis (Mibelli's disease) is a heritable disorder of the skin characterized by localized areas of faulty keratinization, resulting in the cornoid lamella, the morphologic and histologic hall-mark of the disease process. In our review of 35 cases there was little evidence to support the belief that the disease had its origin in the ostia of sweat ducts. Instead the histogenesis of porokeratosis is best explained as a clonal disease of the epidermis much like actinic keratosis. The tendency of these clones to develop is probably inherited. In many cases the latent abnormal clones become clinically overt following actinic exposure.