Porokeratoses constitute a group of rare, clinically and genetically heterogeneous dermatoses characterised by abnormalities in epidermal keratinisation due to pathogenic variants in genes involved in the mevalonate metabolic pathway. Treatment of porokeratosis has long been based on ablative procedures and various topical and systemic therapies, which have demonstrated very limited efficacy. The discovery of the metabolic pathway involved in the pathogenesis paved the way for the development of a new class of topical agents, including a combination of statins and cholesterol, which demonstrate better results compared to previous treatment options. We have described a case of treatment with a topical agent based on a combination of statins and cholesterol for a rare linear form of prokeratosis in an adolescent, which is of considerable value in the context of the differential diagnosis of linear skin lesions, as well as diseases accompanied by impaired keratinisation.

Vestnik dermatologii i venerologii

Вестник дерматологии и венерологии

0042-46092313-6294

Rossijskoe Obschestvo Dermatovenerologov i Kosmetologov

16919

10.25208/vdv16919

CLINICAL CASE REPORTS

НАБЛЮДЕНИЕ ИЗ ПРАКТИКИ

New Therapeutic Opportunities for Linear Porokeratosis

Новые возможности терапии линейного порокератоза

https://orcid.org/0000-0002-8232-8936

4878-5562

Ambarchian

Eduard C

Амбарчян

Эдуард Тигранович

Russian Federation

Head of department pediatric dermatology

Заведующий отделением дерматологии НИИ Педиатрии и охраны здоровья детей

edo_amb@mail.ru

https://orcid.org/0000-0001-5911-553X

2645-4049

Belysheva

Tatiana C

Белышева

Татьяна Сергеевна

klinderma@bk.ru

https://orcid.org/0000-0002-8552-7682

1046-3870

Kletskaya

Irina C

Клецкая

Ирина Семеновна

ikletskaya@gmail.com

https://orcid.org/0000-0003-3003-9398

7014-2239

Alekseeva

Anastasia C

Алексеева

Анастасия Дмитриевна

kuzminova_derma@mail.ru

https://orcid.org/0000-0002-6760-3119

1078-5850

Ivanchikov

Vladislav C

Иванчиков

Владислав Владимирович

Russian Federation

awdawd22@yandex.ru

Petrovsky National Research Centre of SurgeryФедеральное государственное бюджетное научное учреждение "Российский научный центр хирургии имени академика Б.В. Петровского"

Федеральное государственное бюджетное научное учреждение "Российский научный центр хирургии имени академика Б.В. Петровского"

16072026

1023

3006202509062026

Ambarchian E.C., Belysheva T.C., Kletskaya I.C., Alekseeva A.C., Ivanchikov V.C.

Амбарчян Э.Т., Белышева Т.С., Клецкая И.С., Алексеева А.Д., Иванчиков В.В.

https://creativecommons.org/licenses/by-nc/4.0

https://vestnikdv.ru/jour/article/view/16919

Porokeratoses constitute a group of rare, clinically and genetically heterogeneous dermatoses characterised by abnormalities in epidermal keratinisation due to pathogenic variants in genes involved in the mevalonate metabolic pathway. Treatment of porokeratosis has long been based on ablative procedures and various topical and systemic therapies, which have demonstrated very limited efficacy. The discovery of the metabolic pathway involved in the pathogenesis paved the way for the development of a new class of topical agents, including a combination of statins and cholesterol, which demonstrate better results compared to previous treatment options. We have described a case of treatment with a topical agent based on a combination of statins and cholesterol for a rare linear form of prokeratosis in an adolescent, which is of considerable value in the context of the differential diagnosis of linear skin lesions, as well as diseases accompanied by impaired keratinisation.

Порокератозы представляют собой группу редких, клинически и генетически гетерогенных дерматозов, характеризующихся нарушениями эпидермальной кератинизацией из-за патогенных вариантов генов, участвующих в метаболическом пути мевалоната. Лечение порокератозов длительно основывалось на абляционных процедурах и различных топических и системных методиках терапии, которые демонстрировали весьма ограниченную эффективность. Открытие метаболического пути, вовлеченного в патогенез проложило путь к разработке нового класса топических средств, включающих комбинация статинов и холестерина, демонстрирующих лучшие результаты в сравнении с предыдущими вариантами лечения. Нами был описан случай терапии топическим средством на основе комбинации статина и холестерина редкой линейной формы порокератоза у подростка с применением , который представляет значительную ценность в контексте проведения дифференциальной диагностики линейных поражений кожи, а также заболеваний, сопровождающихся нарушением процесса кератинизации. Porokeratosis, linear porokeratosis, pediatrics, dermatology, case reportПорокератоз, линейный порокератоз, педиатрия, дерматология, клинический случай1.Mibelli V. Contributo allo studio della ipercheratosi dei canali sudoriferi (porocheratosi) G Ital Mal Ven Pelle. 1893;28:313–3552.Escanilla-Figueroa C, Jimeno-Ortega I, Fuenzalida-Wong H, Chávez-Rojas F. Generalized linear porokeratosis. An Bras Dermatol. 2018 Jun;93(3):477-478. doi: 10.1590/abd1806-4841.20187798. PMID: 29924219; PMCID: PMC60011063.Nayak MK, Dhanta A, Hazarika N, Kumar A. Linear Systematized Porokeratosis-A Rare Case and Dermoscopic Clues to Diagnosis. Indian Dermatol Online J. 2020 Sep 28;12(3):465-466. doi: 10.4103/idoj.IDOJ_419_20. PMID: 34211921; PMCID: PMC8202469.4.French LE. Christopher Griffiths, Jonathan Barker, Tanya Bleiker, Robert Chalmers, Daniel Creamer., editors. Rook's Textbook of Dermatology. Br J Dermatol. (9th ed) 2017;176:1675–65.Dervis E, Demirkesen C. Generalized linear porokeratosis. Int J Dermatol. 2006;45:1077–9. doi: 10.1111/j.1365-4632.2004.02490.x6.Curkova AK, Hegyi J, Kozub P, Szep Z, D'Erme AM, Simaljakova M. A case of linear porokeratosis treated with photodynamic therapy with confocal microscopy surveillance. Dermatol Ther. 2014 May-Jun;27(3):144-7. doi: 10.1111/dth.12097. Epub 2013 Oct 1. PMID: 241191727.Zhang Z, Li C, Wu F, Ma R, Luan J, Yang F, et al. Genomic variations of the mevalonate pathway in porokeratosis. elife. 2014;4:e063228.Kostopoulos-Kanitakis KA, Kanitakis J. Porokeratoses: an update on pathogenesis and treatment. Int J Dermatol. 2025 Jan;64(1):62-71. doi: 10.1111/ijd.17411. Epub 2024 Aug 11. PMID: 39129190; PMCID: PMC116850609.Kang BY, Jacobsen J, Price HN, Andrews ID. Localized eruptive porokeratosis in pediatric patients following treatment of acute leukemia. Pediatr Dermatol. 2021 Sep;38(5):1226-1232. doi: 10.1111/pde.14772. Epub 2021 Aug 21. PMID: 3441814710.Nazarian RS, Hosseinipour M, Amin B, Cohen SR. Linear porokeratosis presenting in adulthood: A diagnostic challenge: A case report. SAGE Open Med Case Rep. 2020;8 doi: 10.1177/2050313X20919613. 2050313X2091961311.Das A, Vasudevan B, Talwar A. Porokeratosis: An enigma beginning to unravel. Indian J Dermatol Venereol Leprol. 2022;88:291–9. doi: 10.25259/IJDVL_806_2012.Davis CT, Ramani AA, Bharti R. Rapid Progression of Linear Porokeratosis Following COVID-19 Vaccination: A Case Report. Indian Dermatol Online J. 2024 Jul 5;16(1):152-154. doi: 10.4103/idoj.idoj_901_23. PMID: 39850676; PMCID: PMC1175356013.Ф. Знаменская, В.В. Чикин, И.А. Каппушева, В.В. Кондрашова. Диссеминированный поверхностный актинический порокератоз у больного обыкновенным псориазом: клинический случай. Вестник дерматологии и венерологии 2015; (5): 91—96 [F. Znamenskaya, V.V. Chikin, I.A. Kappusheva, V.V. Kondrashova. Disseminated superficial actinic porokeratosis in a patient with common psoriasis: a clinical case study. Bulletin of Dermatology and Venereology 2015; (5): 91-96]14.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 Jan;38(1):242-245. doi: 10.1111/pde.14447. Epub 2020 Nov 10. PMID: 3317051115.Kanitakis J. Porokeratoses: an update of clinical, aetiopathogenic and therapeutic features. Eur J Dermatol 2014; 24: 533–54416.Escanilla-Figueroa C, Jimeno-Ortega I, Fuenzalida-Wong H, Chávez-Rojas F. Generalized linear porokeratosis. An Bras Dermatol. 2018 Jun;93(3):477-478. doi: 10.1590/abd1806-4841.20187798. PMID: 29924219; PMCID: PMC600110617.Winkler JK, Haenssle HA, Enk A, Toberer F. Orange Verrucous Pitted Lesion on Lateral Foot of a 9-year-old Girl: A Quiz. Acta Derm Venereol. 2020 Jul 2;100(14):adv00201. doi: 10.2340/00015555-3544. PMID: 32511737; PMCID: PMC919990318.Pastrana-Ayala R, Peña-Castro GL, Valencia-Herrera AM, Mena-Cedillos CA, Toussaint-Caire S, Akaki-Carreño YI, García-Delgado C, Morán-Barroso VF, Toledo-Bahena M. Craniosynostosis, delayed closure of the fontanelle, anal, genitourinary, and skin abnormalities (CDAGS syndrome): first report in a Mexican patient and review of the literature. Int J Dermatol. 2017 Apr;56(4):435-439. doi: 10.1111/ijd.13504. Epub 2017 Feb 19. PMID: 2821787219.Woźna J, Korecka K, Bowszyc-Dmochowska M, Jałowska M. Porokeratosis of Mibelli Treated With Topical 2% Lovastatin/2% Cholesterol Ointment. Cureus. 2024 Jul 31;16(7):e65871. doi: 10.7759/cureus.65871. PMID: 39219867; PMCID: PMC1136435920.Nguyen CM, Haller CN, Brichta L, Paller AS, Levy ML. Successful treatment of porokeratosis of Mibelli with pathogenesis-directed treatment of topical cholesterol-lovastatin. Pediatr Dermatol. 2024 Nov-Dec;41(6):1253-1254. doi: 10.1111/pde.15657. PMID: 3956362721.Maredia H, Hand JL, Tollefson MM. Pathogenesis-directed treatment of linear porokeratosis with topical cholesterol-lovastatin. Pediatr Dermatol. 2024 Mar-Apr;41(2):296-297. doi: 10.1111/pde.15427. Epub 2023 Sep 19. PMID: 37726979.