Abstract
BackgroundPorokeratosis (PK) is a rare group of keratinization disorders. While the overall prognosis of PK is favorable, malignant transformation of PK to skin cancer has been reported in 6.9% to 11.6% of the cases. Prior estimates of malignant transformation of PK have been based on reviews of published cases, which introduces possible publication bias. We aim to eliminate this potential bias and quantify the characteristics, risk factors, and malignancy potential of PK.MethodologyA single-center retrospective chart review of patients with a diagnosis of PK was conducted.ResultsIn this study, 6.4% to 16.4% of histologically confirmed PK lesions demonstrated malignant transformation. A higher proportion of disseminated superficial actinic porokeratosis (DSAP) cases (as high as 29.3%) showed malignant transformation compared to PK of Mibelli (as high as 6.0%). Out of the two cases of linear PK, both demonstrated malignant transformation.ConclusionsIn summary, PKs are at risk for malignant transformation, and patients with DSAP and linear PK, in particular, should receive more long-term surveillance. Limitations of this study include the inability to control for confounding factors due to the retrospective nature and the small size of our cohort.
Highlights
Porokeratosis (PK) is a rare group of keratinization disorders first described by Vittorio Mibelli in 1889, presenting as annular lesions with an atrophic center and a keratotic ridge [1,2]
A higher proportion of disseminated superficial actinic porokeratosis (DSAP) cases showed malignant transformation compared to PK of Mibelli
PKs are at risk for malignant transformation, and patients with DSAP and linear PK, in particular, should receive more long-term surveillance
Summary
Porokeratosis (PK) is a rare group of keratinization disorders first described by Vittorio Mibelli in 1889, presenting as annular lesions with an atrophic center and a keratotic ridge [1,2]. Malignant transformation of PK to squamous cell carcinoma (SCC) and Bowen’s disease (SCC in situ) have been reported in 6.9% to 11.6% of cases [8,11,12,13,14]. Porokeratosis (PK) is a rare group of keratinization disorders. While the overall prognosis of PK is favorable, malignant transformation of PK to skin cancer has been reported in 6.9% to 11.6% of the cases. Prior estimates of malignant transformation of PK have been based on reviews of published cases, which introduces possible publication bias. We aim to eliminate this potential bias and quantify the characteristics, risk factors, and malignancy potential of PK
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