Malignant Transformation in Porokeratosis Ptychotropica: A Systematic Review
DOI:
https://doi.org/10.2340/actadv.v104.40558Keywords:
porokeratosis, porokeratosis ptychotropica, genitogluteal porokeratosisAbstract
Porokeratosis ptychotropica (PP) is a rare and unusual variant of porokeratosis. There is a dearth of information on the natural history, epidemiology, and optimal treatment options. This study aimed to characterize the worldwide distribution, epidemiology, clinical features, and treatments attempted for all reported cases of porokeratosis ptychotropica. A total of 59 cases of porokeratosis ptychotropica have been reported, with most cases originating from the United States. The median age of patients affected with porokeratosis ptychotropica was 49 years. The most involved body locations are the buttocks and gluteal cleft. The risk of malignant transformation in porokeratosis ptychotropica is approximately 1.7% but there is significant bias in estimating rare occurrences in rare diseases. In conclusion, PP is an important but under-recognized variant of porokeratosis, with a likely low risk of malignant transformation. The best available treatment modality remains uncertain; however, the use of topical lovastatin/cholesterol cream appears promising. Long-term surveillance appears prudent for porokeratosis ptychotropica due to a risk of cancerization.
Downloads
References
Inci R, Zagoras T, Kantere D, Holmström P, Gillstedt M, Polesie S, et al. Porokeratosis is one of the most common genodermatoses and is associated with an increased risk of keratinocyte cancer and melanoma. J Eur Acad Dermatol Venereol 2022; 37: 420-427.
https://doi.org/10.1111/jdv.18587 DOI: https://doi.org/10.1111/jdv.18587
Mazori DR, Shvartsbeyn M, Meehan SA, Tarsis SL. Transformation of porokeratosis ptychotropica into invasive squamous cell carcinoma. Int J Dermatol 2017; 56: 679-680.
https://doi.org/10.1111/ijd.13575 DOI: https://doi.org/10.1111/ijd.13575
Stone N, Ratnavel R, Wilkinson JD. Bilateral perianal inflammatory verrucous porokeratosis (Porokeratosis ptychotropica). Br J Dermatol 1999; 140: 553-555.
https://doi.org/10.1046/j.1365-2133.1999.02738.x DOI: https://doi.org/10.1046/j.1365-2133.1999.02738.x
Pitney L, Weedon D, Pitney M. Porokeratosis ptychotropica: a rare variant with discrete lesions. Australas J Dermatol 2015; 56: e28-29.
https://doi.org/10.1111/ajd.12213 DOI: https://doi.org/10.1111/ajd.12213
Scheiba N, Enk A, Proske S, Hartschuh W. Porokeratosis ptychotropica: successful treatment with the dermatome. Dermatol Surg 2010; 36: 257-260.
https://doi.org/10.1111/j.1524-4725.2009.01402.x DOI: https://doi.org/10.1111/j.1524-4725.2009.01402.x
Tan LS, Chong WS. Porokeratosis in Singapore: an Asian perspective. Australas J Dermatol 2012; 53: e40-44.
https://doi.org/10.1111/j.1440-0960.2011.00856.x DOI: https://doi.org/10.1111/j.1440-0960.2011.00856.x
Atzmony L, Lim YH, Hamilton C, Leventhal JS, Wagner A, Paller AS, et al. Topical cholesterol/lovastatin for the treatment of porokeratosis: a pathogenesis-directed therapy. J Am Acad Dermatol 2020; 82: 123-131.
https://doi.org/10.1016/j.jaad.2019.08.043 DOI: https://doi.org/10.1016/j.jaad.2019.08.043
Santa Lucia G, Snyder A, Lateef A, Drohan A, Gregoski MJ, Barton V, et al. Safety and efficacy of topical lovastatin plus cholesterol cream vs topical lovastatin cream alone for the treatment of disseminated superficial actinic porokeratosis: a randomized clinical trial. JAMA Dermatol 2023; 159: 488-495.
https://doi.org/10.1001/jamadermatol.2023.0205 DOI: https://doi.org/10.1001/jamadermatol.2023.0205
Byth LA, Byth J. Topical simvastatin-cholesterol for disseminated superficial actinic porokeratosis: an open-label, split-body clinical trial. Australas J Dermatol 2021; 62: 310-313.
https://doi.org/10.1111/ajd.13601 DOI: https://doi.org/10.1111/ajd.13601
Diep D, Janitz T, Kannan KS, Crane A, Aluri B, Wright K, et al. Bilateral linear porokeratosis treated with topical cholesterol 2%/lovastatin 2. Cureus 2022; 14: e27540.
https://doi.org/10.7759/cureus.27540 DOI: https://doi.org/10.7759/cureus.27540
Lucker GP, Happle R, Steijlen PM. An unusual case of porokeratosis involving the natal cleft: porokeratosis ptychotropica? Br J Dermatol 1995; 132: 150-151.
https://doi.org/10.1111/j.1365-2133.1995.tb08643.x DOI: https://doi.org/10.1111/j.1365-2133.1995.tb08643.x
Tasker F, Lewis F, Kemp N, Lebas E, Calonje JE. A hidden and rare variant of porokeratosis: a case report of porokeratosis ptychotropica and report of the literature. Australas J Dermatol 2022; 63: 495-496.
https://doi.org/10.1111/ajd.13935 DOI: https://doi.org/10.1111/ajd.13935
Das A, Vasudevan B, Talwar A. Porokeratosis: an enigma beginning to unravel. Indian J Dermatol Venereol Leprol 2022; 88: 291-299.
https://doi.org/10.25259/IJDVL_806_20 DOI: https://doi.org/10.25259/IJDVL_806_20
Hsueh Y-T, Hsu T-C, Hsu C-K, Lee JY-Y, Yang C-C. Disseminated superficial porokeratosis and disseminated superficial actinic porokeratosis: a case series of 39 patients. Dermatologica Sinica 2020; 38: 221-224.
https://doi.org/10.4103/ds.ds_41_20 DOI: https://doi.org/10.4103/ds.ds_41_20
Sasson M, Krain AD. Porokeratosis and cutaneous malignancy: a review. Dermatol Surg 1996; 22: 339-342.
https://doi.org/10.1111/j.1524-4725.1996.tb00327.x DOI: https://doi.org/10.1111/j.1524-4725.1996.tb00327.x
Otsuka F, Someya T, Ishibashi Y. Porokeratosis and malignant skin tumors. J Cancer Res Clin Oncol 1991; 117: 55-60.
https://doi.org/10.1007/BF01613197 DOI: https://doi.org/10.1007/BF01613197
Novice T, Nakamura M, Helfrich Y. The malignancy potential of porokeratosis: a single-center retrospective study. Cureus 2021; 13: e13083.
https://doi.org/10.7759/cureus.13083 DOI: https://doi.org/10.7759/cureus.13083
Maubec E, Duvillard P, Margulis A, Bachollet B, Degois G, Avril MF. Common skin cancers in porokeratosis. Br J Dermatol 2005; 152: 1389-1391.
https://doi.org/10.1111/j.1365-2133.2005.06639.x DOI: https://doi.org/10.1111/j.1365-2133.2005.06639.x
Gu J, Zhang X, Yang Z, Wang N. Expression of cyclin D1 protein isoforms and its prognostic significance in cervical cancer. Cancer Manag Res 2019; 11: 9073-9083.
https://doi.org/10.2147/CMAR.S224026 DOI: https://doi.org/10.2147/CMAR.S224026
Kamata Y, Maejima H, Watarai A, Saito N, Katsuoka K, Takeda A, et al. Expression of bleomycin hydrolase in keratinization disorders. Arch Dermatol Res 2012; 304: 31-38.
https://doi.org/10.1007/s00403-011-1180-6 DOI: https://doi.org/10.1007/s00403-011-1180-6
Knudson AG. Two genetic hits (more or less) to cancer. Nat Rev Cancer 2001; 1: 157-162.
https://doi.org/10.1038/35101031 DOI: https://doi.org/10.1038/35101031
Kubo A, Sasaki T, Suzuki H, Shiohama A, Aoki S, Sato S, et al. Clonal expansion of second-hit cells with somatic recombinations or C>T transitions form porokeratosis in MVD or MVK mutant heterozygotes. J Invest Dermatol 2019; 139: 2458-2466.e59.
https://doi.org/10.1016/j.jid.2019.05.020 DOI: https://doi.org/10.1016/j.jid.2019.05.020
Additional Files
Published
How to Cite
Issue
Section
Categories
License
Copyright (c) 2024 Chee H. Loh, Chris L. Tan, Kong B. Tan, Holger Sudhoff, Peter Goon
This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.
All digitalized ActaDV contents is available freely online. The Society for Publication of Acta Dermato-Venereologica owns the copyright for all material published until volume 88 (2008) and as from volume 89 (2009) the journal has been published fully Open Access, meaning the authors retain copyright to their work.
Unless otherwise specified, all Open Access articles are published under CC-BY-NC licences, allowing third parties to copy and redistribute the material in any medium or format and to remix, transform, and build upon the material for non-commercial purposes, provided proper attribution to the original work.
