Porokeratosis is a disorder of keratinization characterized by one or more atrophic macules or patches surrounded by a distinctive hyperkeratotic ridge-like border called a cornoid lamella (picture 1A-E). Multiple clinical variants of porokeratosis exist. Malignant transformation occurs in a minority of cases.
Although clinical surveillance for malignant transformation is sufficient for the management of most patients with porokeratosis, patients who are concerned about the appearance of lesions or who have associated symptoms such as pruritus or pain may desire therapeutic intervention. Formal studies of the therapeutic options for porokeratosis are lacking, but various topical, excisional, destructive, and systemic therapies appear to be effective in some patients.
The pathogenesis, clinical manifestations, diagnosis, and management of porokeratosis will be reviewed here.
The defining feature of porokeratosis is the clinical and histopathologic presence of a cornoid lamella, which typically manifests as a thin keratotic rim at the periphery of a slightly atrophic skin lesion. The most commonly described variants of porokeratosis include:
●Disseminated superficial actinic porokeratosis (DSAP, OMIM #175900, #607728, #612293, and #612353)