Familial porokeratosis of Mibelli: a peerless entity

ABSTRACT

Porokeratosis are a group of hereditary or acquired disorders characterized by annular plaques with an atrophic centre and a hyperkeratotic peripheral ridge. Pathologically, porokeratosis is characterized by a column of parakeratotic cells, called the cornoid lamella. This report describes a 55-year-old male with his two sons affected by porokeratosis of Mibelli and it was confirmed histopathologically. The rarity of this disorder in family, its clinical exuberance and the destructive character of the lesions, as well as, nasal, finger and abdomen involvement were unusual in case of porokeratosis