BACKGROUND. Porokeratosis is a disorder of epidermal keratinization ma
nifested clinically by a raised border and histologically by a cornoid
lamella. While cutaneous malignancy has been reported to arise in por
okeratosis, the risk remains unknown. In the past it has been associat
ed with a history of radiation therapy. OBJECTIVE. The purpose of this
study is to estimate the frequency of cutaneous malignancy arising in
porokeratosis and define those patients who are at highest risk. METH
ODS. All cases in the English language literature in the last 30 years
were reviewed. RESULTS. Twenty-one (7.5%) of 281 cases reported revea
led a malignancy arising within porokeratosis. Large lesions, those of
long-standing duration, and the linear type were at greatest risk. Ra
diation therapy was an infrequently identified risk factor. CONCLUSION
. Porokeratosis is a premalignant condition, with certain groups of pa
tients at greatest risk for malignant transformation.