Background: Porokeratosis (PK) is a rare skin disease of unknown etiology. It consists of a keratinization disorder, which may appear in several clinical forms and can undergo malignant transformation. The histopathological hallmark of PK is the cornoid lamella. While many topical, systemic, and surgical treatment modalities for PK have been described, no randomized controlled trials have been performed yet. Because of a lack of treatment standards for PK, European and international guidelines cannot be created.
Objective: The aim of this systematic review is to outline options for treating PK.
Methods: We performed a systematic literature search in an electronic database for published literature. A total of 88 articles fulfilling our inclusion criteria were found.
Results: There were no randomized controlled trials on the treatment of PK, but mainly case reports and case series. Porokeratosis of Mibelli showed the best outcomes after treatment with imiquimod cream and linear PK responded well to topical or systemic retinoids. Topical vitamin D acid derivatives may be the best therapeutic option for disseminated PK. Surgical interventions and cryotherapy may be preferred in areas where the use of topical agents is difficult or contraindicated.
Conclusion: To offer patients with PK an evidence-based high-quality standardized therapy, randomized controlled trials are needed.