A Review of Therapeutic Options for Linear Porokeratosis
Main Article Content
Keywords
linear porokeratosis, management, topical therapies, cryotherapy, photodynamic therapies, laser therapies, surgical procedures, systemic therapies
Abstract
Linear porokeratosis (LP) is a rare and uncommon variant of porokeratosis presenting as linear annular papules or plaques along Blaschko’s lines. It may arise spontaneously, although evidence of genetic predisposition exists via point mutations in the mevalonate pathway of cholesterol biosynthesis. Malignant transformation is the most serious complication of this chronic, progressive dyskeratosis. We review the currently available treatment options for LP, including topical, systemic, laser, photodynamic, and surgical therapies.
References
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5. Saleva-Stateva M, Hess M, Technau-Hafsi K, et al. Molecular characterization and natural history of linear porokeratosis: A case series. J Am Acad Dermatol. 2021;85(6):1603-1606. doi: 10.1016/j.jaad.2020.11.061
6. Happle R. Cancer proneness of linear porokeratosis may be explained by allelic loss. Dermatology. 1997;195(1):20-5. doi: 10.1159/000245678.
7. Weidner T, Illing T, Miguel D, Elsner P. Treatment of porokeratosis: a systematic review. Am J Clin Dermatol. 2017;18(4):435-449. doi:10.1007/s40257-017-0271-3
8. Sasaki S, Urano Y, Nakagawa K, et al. Linear porokeratosis with multiple squamous cell carcinomas: study of p53 expression in porokeratosis and squamous cell carcinoma. Br J Dermatol. 1996;134(6):1151-1153. doi: 10.1111/j.1365-2133.1996.tb07965.x
9. Balasubramanian S, Mangalampalli VR, Vasudevan B. Linear porokeratosis: an uncommon presentation of a common condition. Indian J Dermatol. 2017;62(6):632-634.
10. Pels R, Sterry W, Lademann J. Clobetasol propionate--where, when, why?. Drugs Today (Barc). 2008;44(7):547-557. doi:10.1358/dot.2008.44.7.1122221
11. Parks AC, Conner KJ, Armstrong CA. Long-term clearance of linear porokeratosis with tacrolimus, 0.1%, ointment. JAMA Dermatol. 2014;150(2):194-196. doi:10.1001/jamadermatol.2013.8526
12. Ahn SJ, Lee HJ, Chang SE, et al. Case of linear porokeratosis: successful treatment with topical 5% imiquimod cream. J Dermatol. 2007;34(2):146–7. doi:10.1111/j.1346-8138.2006.00236.x
13. Zasada M, Budzisz E. Retinoids: active molecules influencing skin structure formation in cosmetic and dermatological treatments. Postepy Dermatol Alergol. 2019;36(4):392-397. doi:10.5114/ada.2019.87443
14. Grover C, Goel A, Nanda S, et al. A case of extensive linear porokeratosis with evaluation of topical tretinoin versus 5-fluorouracil as treatment modalities. J Dermatol. 2005;32(12):1000-1004. doi:10.1111/j.1346-8138.2005.tb00890.x
15. Dervis E, Demirkesen C. Generalized linear porokeratosis. Int J Dermatol. 2006;45(9):1077–9. doi:10.1111/j.1365-4632.2004.02490.x
16. Balato N, Di Nardo G, Boccia L, Nappa P. [Linear Mibelli's porokeratosis. Treatment with topical 5-fluorouracil]. G Ital Dermatol Venereol. 1986;121(2):147-148.
17. Kohara Y, Takeo T, Oshima Y, et al. Linear porokeratosis with nail dystrophy. Eur J Dermatol. 2011;21(4):625–6. doi:10.1684/ejd.2011.1393
18. Yang J, Du YQ, Fang XY, et al. Linear porokeratosis of the foot with dermoscopic manifestations: a case report. World J Clin Cases. 2022;10(31):11585-11589. doi:10.12998/wjcc.v10.i31.11585
19. Atzmony L, Lim YH, Hamilton C, et al. Topical cholesterol/lovastatin for the treatment of porokeratosis: a pathogenesis-directed therapy. J Am Acad Dermatol. 2020;82(1):123-131. doi: 10.1016/j.jaad.2019.08.043
20. Buhle AC, Fagan KK, Johnson NM, Grider DJ. Treating linear porokeratosis with topical lovastatin/cholesterol cream. Dermatol Online J. 2022 Oct 15;28(5). doi: 10.5070/D328559247
21. Diep D, Janitz T, Kannan KS, Crane A, Aluri B, Wright K, Baker W. Bilateral linear porokeratosis treated with topical cholesterol 2%/lovastatin 2. Cureus. 2022 Jul 31;14(7):e27540. doi: 10.7759/cureus.27540
22. Blue E, Abbott J, Bowen A, Cipriano SD. Linear porokeratosis with bone abnormalities treated with compounded topical 2% cholesterol/2% lovastatin ointment. Pediatr Dermatol. 2021;38(1):242-245. doi: 10.1111/pde.14447
23. Saleva-Stateva M, Weibel L, Theiler M, Balabanova M, Boente MC, Has C. Lack of effect of topical statins in linear porokeratosis. J Eur Acad Dermatol Venereol. 2021;35(1):e26-e28. doi: 10.1111/jdv.16768
24. Bhushan M, Craven NM, Beck MH, Chalmers RJ. Linear porokeratosis of mibelli: successful treatment with cryotherapy. Br J Dermatol. 1999;141(2):389. doi: 10.1046/j.1365-2133.1999.03023.x
25. Calzavara-Pinton PG, Rossi MT, Aronson E, Sala R; Italian Group For Photodynamic Therapy. A retrospective analysis of real-life practice of off-label photodynamic therapy using methyl aminolevulinate (MAL-PDT) in 20 Italian dermatology departments. Part 1: inflammatory and aesthetic indications. Photochem Photobiol Sci. 2013;12(1):148-57. doi: 10.1039/c2pp25124h
26. Curkova AK, Hegyi J, Kozub P, et al. A case of linear porokeratosis treated with photodynamic therapy with confocal microscopy surveillance. Dermatol Ther. 2014;27(3):144-147. doi:10.1111/dth.12097
27. García-Navarro X, Garcés JR, Baselga E, Alomar A. Linear porokeratosis: excellent response to photodynamic therapy. Arch Dermatol. 2009;145(5):526-527. doi:10.1001/archdermatol.2009.45
28. Garrido-Colmenero C, Ruiz-Villaverde R, Martínez-García E, Aneiros-Fernández J, Tercedor-Sánchez J. Photoletter to the editor: Response of linear porokeratosis to photodynamic therapy in an 11-year-old girl. J Dermatol Case Rep. 2015;9(4):118-119. doi:10.3315/jdcr.2015.1220
29. Bernstein EF. The pulsed-dye laser for treatment of cutaneous conditions. G Ital Dermatol Venereol. 2009;144(5):557-572.
30. Alster TS, Nanni CA. Successful treatment of porokeratosis with 585 nm pulsed dye laser irradiation. Cutis. 1999;63(5):265-266.
31. Husain Z, Alster TS. The role of lasers and intense pulsed light technology in dermatology. Clin Cosmet Investig Dermatol. 2016;9:29-40. doi:10.2147/CCID.S69106
32. Casale F, Tchanque-Fossuo CN, Durkin JR. Successful clearance of linear porokeratosis with aminolevulinic acid and pulsed dye laser. Dermatol Surg. 2021;47(9):1300-1301. doi: 10.1097/DSS.0000000000002996
33. Barnett JH. Linear porokeratosis: treatment with the carbon dioxide laser. J Am Acad Dermatol. 1986;14(5 Pt 2):902-904. doi:10.1016/s0190-9622(86)70108-4
34. Cohen PR, Held JL, Katz BE. Linear porokeratosis: successful treatment with diamond fraise dermabrasion. J Am Acad Dermatol. 1990;23(5 Pt 2):975-7. doi: 10.1016/0190-9622(90)70317-b
35. Abbott-Frey AM, Coromilas AJ, Niedt GW, Lewin JM. Recurrent squamous cell carcinoma arising within a linear porokeratosis. J Drugs Dermatol. 2020;19(2):205-206. doi:10.36849/JDD.2020.4640
36. Sommerlad M, Lock A, Moir G, McGregor J, Bull R, Cerio R, Harwood C. Linear porokeratosis with multiple squamous cell carcinomas successfully treated by electrochemotherapy. Br J Dermatol. 2016;175(6):1342-1345. doi: 10.1111/bjd.14669
37. Hong JB, Hsiao CH, Chu CY. Systematized linear porokeratosis: a rare variant of diffuse porokeratosis with good response to systemic acitretin. J Am Acad Dermatol. 2009;60(4):713-5. doi: 10.1016/j.jaad.2008.10.011
38. Pehamberger H, Konrad K. Treatment with an oral aromatic retinoid in linear porokeratosis. Dermatologica. 1980;160(4):270-4. doi: 10.1159/000250503
2. Nayak MK, Dhanta A, Hazarika N, Kumar A. Linear systematized porokeratosis - a rare case and dermoscopic clues to diagnosis. Indian Dermatol Online J. 2020;12(3):465-466. doi:10.4103/idoj.IDOJ_419_20
3. Atzmony L, Khan HM, Lim YH, Paller AS, Levinsohn JL, Holland KE, Mirza FN, Yin E, Ko CJ, Leventhal JS, Choate KA. Second-Hit, Postzygotic PMVK and MVD Mutations in Linear Porokeratosis. JAMA Dermatol. 2019 May 1;155(5):548-555. doi: 10.1001/jamadermatol.2019.0016
4. Zhang Z, Li C, Wu F, et al. Genomic variations of the mevalonate pathway in porokeratosis. Elife. 2015;4:e06322. doi:10.7554/eLife.06322
5. Saleva-Stateva M, Hess M, Technau-Hafsi K, et al. Molecular characterization and natural history of linear porokeratosis: A case series. J Am Acad Dermatol. 2021;85(6):1603-1606. doi: 10.1016/j.jaad.2020.11.061
6. Happle R. Cancer proneness of linear porokeratosis may be explained by allelic loss. Dermatology. 1997;195(1):20-5. doi: 10.1159/000245678.
7. Weidner T, Illing T, Miguel D, Elsner P. Treatment of porokeratosis: a systematic review. Am J Clin Dermatol. 2017;18(4):435-449. doi:10.1007/s40257-017-0271-3
8. Sasaki S, Urano Y, Nakagawa K, et al. Linear porokeratosis with multiple squamous cell carcinomas: study of p53 expression in porokeratosis and squamous cell carcinoma. Br J Dermatol. 1996;134(6):1151-1153. doi: 10.1111/j.1365-2133.1996.tb07965.x
9. Balasubramanian S, Mangalampalli VR, Vasudevan B. Linear porokeratosis: an uncommon presentation of a common condition. Indian J Dermatol. 2017;62(6):632-634.
10. Pels R, Sterry W, Lademann J. Clobetasol propionate--where, when, why?. Drugs Today (Barc). 2008;44(7):547-557. doi:10.1358/dot.2008.44.7.1122221
11. Parks AC, Conner KJ, Armstrong CA. Long-term clearance of linear porokeratosis with tacrolimus, 0.1%, ointment. JAMA Dermatol. 2014;150(2):194-196. doi:10.1001/jamadermatol.2013.8526
12. Ahn SJ, Lee HJ, Chang SE, et al. Case of linear porokeratosis: successful treatment with topical 5% imiquimod cream. J Dermatol. 2007;34(2):146–7. doi:10.1111/j.1346-8138.2006.00236.x
13. Zasada M, Budzisz E. Retinoids: active molecules influencing skin structure formation in cosmetic and dermatological treatments. Postepy Dermatol Alergol. 2019;36(4):392-397. doi:10.5114/ada.2019.87443
14. Grover C, Goel A, Nanda S, et al. A case of extensive linear porokeratosis with evaluation of topical tretinoin versus 5-fluorouracil as treatment modalities. J Dermatol. 2005;32(12):1000-1004. doi:10.1111/j.1346-8138.2005.tb00890.x
15. Dervis E, Demirkesen C. Generalized linear porokeratosis. Int J Dermatol. 2006;45(9):1077–9. doi:10.1111/j.1365-4632.2004.02490.x
16. Balato N, Di Nardo G, Boccia L, Nappa P. [Linear Mibelli's porokeratosis. Treatment with topical 5-fluorouracil]. G Ital Dermatol Venereol. 1986;121(2):147-148.
17. Kohara Y, Takeo T, Oshima Y, et al. Linear porokeratosis with nail dystrophy. Eur J Dermatol. 2011;21(4):625–6. doi:10.1684/ejd.2011.1393
18. Yang J, Du YQ, Fang XY, et al. Linear porokeratosis of the foot with dermoscopic manifestations: a case report. World J Clin Cases. 2022;10(31):11585-11589. doi:10.12998/wjcc.v10.i31.11585
19. Atzmony L, Lim YH, Hamilton C, et al. Topical cholesterol/lovastatin for the treatment of porokeratosis: a pathogenesis-directed therapy. J Am Acad Dermatol. 2020;82(1):123-131. doi: 10.1016/j.jaad.2019.08.043
20. Buhle AC, Fagan KK, Johnson NM, Grider DJ. Treating linear porokeratosis with topical lovastatin/cholesterol cream. Dermatol Online J. 2022 Oct 15;28(5). doi: 10.5070/D328559247
21. Diep D, Janitz T, Kannan KS, Crane A, Aluri B, Wright K, Baker W. Bilateral linear porokeratosis treated with topical cholesterol 2%/lovastatin 2. Cureus. 2022 Jul 31;14(7):e27540. doi: 10.7759/cureus.27540
22. Blue E, Abbott J, Bowen A, Cipriano SD. Linear porokeratosis with bone abnormalities treated with compounded topical 2% cholesterol/2% lovastatin ointment. Pediatr Dermatol. 2021;38(1):242-245. doi: 10.1111/pde.14447
23. Saleva-Stateva M, Weibel L, Theiler M, Balabanova M, Boente MC, Has C. Lack of effect of topical statins in linear porokeratosis. J Eur Acad Dermatol Venereol. 2021;35(1):e26-e28. doi: 10.1111/jdv.16768
24. Bhushan M, Craven NM, Beck MH, Chalmers RJ. Linear porokeratosis of mibelli: successful treatment with cryotherapy. Br J Dermatol. 1999;141(2):389. doi: 10.1046/j.1365-2133.1999.03023.x
25. Calzavara-Pinton PG, Rossi MT, Aronson E, Sala R; Italian Group For Photodynamic Therapy. A retrospective analysis of real-life practice of off-label photodynamic therapy using methyl aminolevulinate (MAL-PDT) in 20 Italian dermatology departments. Part 1: inflammatory and aesthetic indications. Photochem Photobiol Sci. 2013;12(1):148-57. doi: 10.1039/c2pp25124h
26. Curkova AK, Hegyi J, Kozub P, et al. A case of linear porokeratosis treated with photodynamic therapy with confocal microscopy surveillance. Dermatol Ther. 2014;27(3):144-147. doi:10.1111/dth.12097
27. García-Navarro X, Garcés JR, Baselga E, Alomar A. Linear porokeratosis: excellent response to photodynamic therapy. Arch Dermatol. 2009;145(5):526-527. doi:10.1001/archdermatol.2009.45
28. Garrido-Colmenero C, Ruiz-Villaverde R, Martínez-García E, Aneiros-Fernández J, Tercedor-Sánchez J. Photoletter to the editor: Response of linear porokeratosis to photodynamic therapy in an 11-year-old girl. J Dermatol Case Rep. 2015;9(4):118-119. doi:10.3315/jdcr.2015.1220
29. Bernstein EF. The pulsed-dye laser for treatment of cutaneous conditions. G Ital Dermatol Venereol. 2009;144(5):557-572.
30. Alster TS, Nanni CA. Successful treatment of porokeratosis with 585 nm pulsed dye laser irradiation. Cutis. 1999;63(5):265-266.
31. Husain Z, Alster TS. The role of lasers and intense pulsed light technology in dermatology. Clin Cosmet Investig Dermatol. 2016;9:29-40. doi:10.2147/CCID.S69106
32. Casale F, Tchanque-Fossuo CN, Durkin JR. Successful clearance of linear porokeratosis with aminolevulinic acid and pulsed dye laser. Dermatol Surg. 2021;47(9):1300-1301. doi: 10.1097/DSS.0000000000002996
33. Barnett JH. Linear porokeratosis: treatment with the carbon dioxide laser. J Am Acad Dermatol. 1986;14(5 Pt 2):902-904. doi:10.1016/s0190-9622(86)70108-4
34. Cohen PR, Held JL, Katz BE. Linear porokeratosis: successful treatment with diamond fraise dermabrasion. J Am Acad Dermatol. 1990;23(5 Pt 2):975-7. doi: 10.1016/0190-9622(90)70317-b
35. Abbott-Frey AM, Coromilas AJ, Niedt GW, Lewin JM. Recurrent squamous cell carcinoma arising within a linear porokeratosis. J Drugs Dermatol. 2020;19(2):205-206. doi:10.36849/JDD.2020.4640
36. Sommerlad M, Lock A, Moir G, McGregor J, Bull R, Cerio R, Harwood C. Linear porokeratosis with multiple squamous cell carcinomas successfully treated by electrochemotherapy. Br J Dermatol. 2016;175(6):1342-1345. doi: 10.1111/bjd.14669
37. Hong JB, Hsiao CH, Chu CY. Systematized linear porokeratosis: a rare variant of diffuse porokeratosis with good response to systemic acitretin. J Am Acad Dermatol. 2009;60(4):713-5. doi: 10.1016/j.jaad.2008.10.011
38. Pehamberger H, Konrad K. Treatment with an oral aromatic retinoid in linear porokeratosis. Dermatologica. 1980;160(4):270-4. doi: 10.1159/000250503
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Published
May 13, 2024
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Verma, K., M. K. Shalabi, M. ., C. Hartmann, A., & P. Friedmann, D. (2024). A Review of Therapeutic Options for Linear Porokeratosis. SKIN The Journal of Cutaneous Medicine, 8(3), 1500–1505. https://doi.org/10.25251/skin.8.3.3
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In-Depth Reviews
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