Porokeratosis Mibelli gigantea: case report and literature review

Porokeratos of Mibelli is a rare inherited disorder of epidermal keratiniza tion, whose pathogenesis is not fully understood. The common clinical featu re is a erythematous plaque surrounded by a hyperkeratotic border. The hist opathologic 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.