[Porokeratosis Mibelli gigantea: case report and literature review]

Abstract

Porokeratos of Mibelli is a rare inherited disorder of epidermal keratinization, whose pathogenesis is not fully understood. The common clinical feature is a erythematous plaque surrounded by a hyperkeratotic border. The histopathologic hallmark is a parakeratotic cornoid lamella. An unusual case of porokeratosis gigantea, a morphological variant of classical porokeratosis of Mibelli, is reported. The pathogenesis, taking in account especially the clonal hypothesis and premalignant nature of porokeratosis, is discussed.