Красный волосяной лишай (КВЛ, син.: болезнь Девержи, красный волосяной отрубевидный лишай, питириаз красный волосяной отрубевидный, pityriasis rubra pilaris) – редкое идиопатическое папулезно-сквамозное воспалительное заболевание кожи, характеризующееся фолликулярными роговыми папулами, ладонно-подошвенной кератодермией, шелушащимися бляшками оранжево-красного цвета с характерными очагами непораженной кожи. Гистологически чередующийся паттерн орто- и паракератоза считается отличительной чертой КВЛ (шахматный гиперкератоз). Нарушение регуляции врожденного иммунитета является компонентом патогенеза КВЛ. Врожденные мутации гена CARD14 или сопутствующие антигены, которые могут принимать форму ятрогенных поражений, инфекций или злокачественных новообразований, могут быть начальными пусковыми механизмами заболевания, хотя этиология часто идиопатическая. Классически подразделяют 5 подтипов заболевания в зависимости от возраста начала и клинической картины. КВЛ VI типа ассоциирован с инфекцией, вызванной вирусом иммунодефицита человека. CARD14-ассоциированная папулезно-сквамозная сыпь и дискоидный дерматит лица представляют собой новые клинические фенотипы КВЛ. Последний оказывает выраженное негативное влияние на качество жизни, а заболевшие подвержены повышенному риску депрессии и суициду. Иксекизумаб, метотрексат и секукинумаб могут рассматриваться как системные препараты 1-го выбора. При наличии противопоказания к иммуносупрессивной терапи рекомендуется прием высоких доз изотретиноина. В качестве дополнительной терапии применяются местные средства, включая кальципотриен, ингибиторы кальциневрина, смягчающие средства и топические кортикостероиды.
Pityriasis rubra pilaris (PRP, syn.: Devergie's disease) is a rare idiopathic papulo-squamous inflammatory skin disease characterized by follicular papules, palmar-plantar keratoderma, scaling plaques of orange-red color with characteristic foci of unaffected skin. Histologically, the alternating pattern of orthokeratosis and parakeratosis is considered a distinctive feature of PRP (staggered hyperkeratosis). A violation of the regulation of innate immunity is a component of the pathogenesis of PRP. Congenital mutations of CARD 14 or concomitant antigens, which can take the form of iatrogenic lesions, infections or malignant neoplasms, can be the initial triggers of the disease, although the etiology is often idiopathic. There are classically five subtypes of the disease, depending on the age of onset and clinical picture. Type VI PRP is associated with infection caused by the human immunodeficiency virus. CARD 14-associated papulosquamous rash and discoid dermatitis of the face represent new clinical phenotypes of PRP. Devergie's disease has a pronounced negative impact on the quality of life, and those who become ill are at increased risk of depression and suicide. Ixekizumab, methotrexate and secukinumab can be considered as systemic drugs of the first choice. If there is a contraindication to immunosuppressive therapy, high doses of isotretinoin are recommended. Topical agents, including calcipotriene, calcineurin inhibitors, emollients and topical corticosteroids, are used as additional therapy.
1. Joshi TP, Duvic M. Pityriasis rubra pilaris: An updated review of clinical presentation, etiopathogenesis, and treatment options. Am J Clin Dermatol. 2024;25(2):243-59. DOI:10.1007/s40257-023-00836-x
2. Greiling TM, Brown F, Syed HA. Pityriasis rubra pilaris. In: StatPearls. Treasure Island (FL): StatPearls Publishing, 2024.
3. Brown F, Badri T. Pityriasis rubra pilaris. In: StatPearls. Treasure Island (FL): StatPearls Publishing, 2023. Available at: https://www.ncbi.nlm.nih.gov/books/NBK482436. Accessed: 23.05.2024.
4. Zhou T, Al Muqrin A, Abu-Hilal M. Updates on pityriasis rubra pilaris: A scoping review. J Cutan Med Surg. 2024;28(2):158-66. DOI:10.1177/12034754231223159
5. Bertin J, Wang L, Guo Y, et al. CARD11 and CARD14 are novel caspase recruitment domain (CARD)/membrane-associated guanylate kinase (MAGUK) family members that interact with BCL10 and activate NF-kappa B. J Biol Chem. 2001;276(15):11877-82. DOI:10.1074/jbc.M010512200
6. Scudiero I, Zotti T, Ferravante A, et al. Alternative splicing of CARMA2/CARD14 transcripts generates protein variants with differential effect on NF-κB activation and endoplasmic reticulum stress-induced cell death. J Cell Physiol. 2011;226(12):3121-31. DOI:10.1002/jcp.22667
7. Craiglow BG, Boyden LM, Hu R, et al. CARD14-associated papulosquamous eruption: A spectrum including features of psoriasis and pityriasis rubra pilaris. J Am Acad Dermatol. 2018;79(3):487-94. DOI:10.1016/j.jaad.2018.02.034
8. Silva JA, de Carvalho Mesquita K, Igreja AC, et al. Paraneoplastic cutaneous manifestations: concepts and updates. An Bras Dermatol. 2013;88(1):9-22.
DOI:10.1590/s0365-05962013000100001
9. Griffiths WA. Pityriasis rubra pilaris. Clin Exp Dermatol. 1980;5:105-12. DOI:10.1111/j.1365-2230.1980.tb01676.x
10. Ross NA, Chung HJ, Li Q, et al. Epidemiologic, clinicopathologic, diagnostic, and management challenges of pityriasis rubra pilaris: A case series of 100 patients. JAMA Dermatol. 2016;152(6):670-5. DOI:10.1001/jamadermatol.2016.0091
11. Ji-Xu A, Lei DK, Maloney NJ, Worswick S. Clinical course, diagnostic patterns, and treatment outcomes in patients with pityriasis rubra pilaris. J Am Acad Dermatol.
2022;87(6):1450-1. DOI:10.1016/j.jaad.2022.07.039
12. Ghatnekar S, Shah N, Min MS, et al. Clinical features and eosinophilia in pityriasis rubra pilaris: A multicenter cohort. J Am Acad Dermatol. 2022;86:907-9. DOI:10.1016/j.jaad.2021.03.043
13. Yang CC, Shih IH, Lin WL, et al. Juvenile pityriasis rubra pilaris: Report of 28 cases in Taiwan. J Am Acad Dermatol. 2008;59(6):943-8. DOI:10.1016/j.jaad.2008.07.054
14. Allison DS, El-Azhary RA, Calobrisi SD, Dicken CH. Pityriasis rubra pilaris in children. J Am Acad Dermatol. 2002;47(3):386-9. DOI:10.1067/mjd.2002.124619
15. Blauvelt A, Nahass GT, Pardo RJ, Kerdel FA. Pityriasis rubra pilaris and HIV infection. J Am Acad Dermatol. 1991;24 24(5 Pt 1):703-5. DOI:10.1016/0190-9622(91)70106-c
16. Miralles ES, Núñez M, De Las Heras ME, et al. Pityriasis rubra pilaris and human immunodefciency virus infection. Br J Dermatol. 1995; 133(6):990-3.
DOI:10.1111/j.1365-2133.1995.tb06939.x
17. Nair PA, Sheth N. Atypical adult-onset pityriasis rubra pilaris in an HIV-positive adult male. Indian J Dermatol. 2018;63(6):522-4. DOI:10.4103/ijd.IJD_141_18
18. Kranyak A, Shuler M. Pityriasis rubra pilaris rapidly cleared with Ixekizumab in an HIV-positive patient. JAAD Case Rep. 2022;27:55-7. DOI:10.1016/j.jdcr.2022.07.015
19. Williams A, George A, Thomas EA, Koshy JM. Pityriasis rubra pilaris type 6: A case report in an AIDS patient. Indian J Sex Transm Dis AIDS. 2020;41(1):100-1. DOI:10.4103/ijstd.IJSTD_120_15
20. Allegue F, Fachal C, Iglesias B, Zulaica A. Facial discoid dermatosis: a new variant of pityriasis rubra pilaris? Actas Dermosifiliogr. 2022;113(7):728-31. DOI:10.1016/j.ad.2021.01.006
21. Li N, Tao J, Zhang J, et al. A novel mutation in a CARD14-associated papulosquamous eruption. Pediatr Dermatol. 2023;40(4):706-9. DOI:10.1111/pde.15254
22. Takeichi T, Terawaki S, Kubota Y, et al. A patient with CARD14-associated papulosquamous eruptions showing atopic dermatitis-like features. J Eur Acad Dermatol Venereol. 2021;35(1):e58-9. DOI:10.1111/jdv.16799
23. Ko CJ, Heald P, Antaya RJ, Bolognia JL. Facial discoid dermatosis. Int J Dermatol. 2010;49(2):189-92. DOI:10.1111/j.1365-4632.2009.04206.x
24. Salman A, Tekin B, Berenjian A, et al. Facial discoid dermatosis: A further case of a novel entity. J Dermatol. 2015;42(11):1132-3. DOI:10.1111/1346-8138.13045
25. Gan EY, Ng SK, Goh CL, Lee SSJ. Recalcitrant psoriasiform dermatosis of the face: is it related to pityriasis rubra pilaris? J Cutan Pathol. 2018;45(7):491-7. DOI:10.1111/cup.13148
26. Rypka KJ, Fulk TS, Afsaneh A, et al. Improvement of facial discoid dermatosis with ustekinumab treatment. JAMA Dermatol. 2022;158(9):1079-80. DOI:10.1001/jamadermatol.2022.2478
27. Davis AE, Raine BE, Swartzman I, et al. Rethinking pityriasis rubra pilaris as a paraneoplastic syndrome: two cases of pityriasis rubra pilaris with concomitant underlying malignancy. JAAD Case Rep. 2023;32:90-5. DOI:10.1016/j.jdcr.2022.12.007
28. Roenneberg S, Biedermann T. Pityriasis rubra pilaris: algorithms for diagnosis and treatment. J Eur Acad Dermatol Venereol. 2018;32(6):889-98. DOI:10.1111/jdv.14761
29. Naidoo A, Sibbald C, Fleming PJ, Piguet V. Use of biologics in pityriasis rubra pilaris refractory to first-line systemic therapy: A systematic review. J Cutan Med Surg. 2020;24(1):73-8. DOI:10.1177/1203475419887731
30. Kromer C, Sabat R, Celis D, Mössner R. Systemic therapies of pityriasis rubra pilaris: A systematic review. J Dtsch Dermatol Ges. 2019;17(3):243-59. DOI:10.1111/ddg.13718
31. Wassef C, Lombardi A, Rao BK. Adalimumab for the treatment of pityriasis rubra pilaris: A case report. Cutis. 2012;90(5):244-7.
32. Wohlrab J, Kreft B. Treatment of pityriasis rubra pilaris with ustekinumab. Br J Dermatol 2010;163(3):655-6. DOI:10.1111/j.1365-2133.2010.09855.x
33. Napolitano M, Lembo L, Fania L, et al. Ustekinumab treatment of pityriasis rubra pilaris: A report of five cases. J Dermatol. 2018;45(2):202-6. DOI:10.1111/1346-8138.14114
34. Haynes D, Strunck JL, Topham CA, et al. Evaluation of ixekizumab treatment for patients with pityriasis rubra pilaris: A single-arm trial. JAMA Dermatol. 2020;156(6):668-75. DOI:10.1001/jamadermatol.2020.0932
35. Boudreaux BW, Pincelli TP, Bhullar PK, et al. Secukinumab for the treatment of adult-onset pityriasis rubra pilaris: A single-arm clinical trial with transcriptomic analysis. Br J Dermatol. 2022;187(5):650-8. DOI:10.1111/bjd.21708
36. Ji-Xu A, Lei DK, Worswick S, et al. Patient and disease characteristics associated with psychiatric symptoms and impaired quality of life in pityriasis rubra pilaris. Br J Dermatol. 2022;187(6):1024-6. DOI:10.1111/bjd.21792
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1. Joshi TP, Duvic M. Pityriasis rubra pilaris: An updated review of clinical presentation, etiopathogenesis, and treatment options. Am J Clin Dermatol. 2024;25(2):243-59. DOI:10.1007/s40257-023-00836-x
2. Greiling TM, Brown F, Syed HA. Pityriasis rubra pilaris. In: StatPearls. Treasure Island (FL): StatPearls Publishing, 2024.
3. Brown F, Badri T. Pityriasis rubra pilaris. In: StatPearls. Treasure Island (FL): StatPearls Publishing, 2023. Available at: https://www.ncbi.nlm.nih.gov/books/NBK482436. Accessed: 23.05.2024.
4. Zhou T, Al Muqrin A, Abu-Hilal M. Updates on pityriasis rubra pilaris: A scoping review. J Cutan Med Surg. 2024;28(2):158-66. DOI:10.1177/12034754231223159
5. Bertin J, Wang L, Guo Y, et al. CARD11 and CARD14 are novel caspase recruitment domain (CARD)/membrane-associated guanylate kinase (MAGUK) family members that interact with BCL10 and activate NF-kappa B. J Biol Chem. 2001;276(15):11877-82. DOI:10.1074/jbc.M010512200
6. Scudiero I, Zotti T, Ferravante A, et al. Alternative splicing of CARMA2/CARD14 transcripts generates protein variants with differential effect on NF-κB activation and endoplasmic reticulum stress-induced cell death. J Cell Physiol. 2011;226(12):3121-31. DOI:10.1002/jcp.22667
7. Craiglow BG, Boyden LM, Hu R, et al. CARD14-associated papulosquamous eruption: A spectrum including features of psoriasis and pityriasis rubra pilaris. J Am Acad Dermatol. 2018;79(3):487-94. DOI:10.1016/j.jaad.2018.02.034
8. Silva JA, de Carvalho Mesquita K, Igreja AC, et al. Paraneoplastic cutaneous manifestations: concepts and updates. An Bras Dermatol. 2013;88(1):9-22.
DOI:10.1590/s0365-05962013000100001
9. Griffiths WA. Pityriasis rubra pilaris. Clin Exp Dermatol. 1980;5:105-12. DOI:10.1111/j.1365-2230.1980.tb01676.x
10. Ross NA, Chung HJ, Li Q, et al. Epidemiologic, clinicopathologic, diagnostic, and management challenges of pityriasis rubra pilaris: A case series of 100 patients. JAMA Dermatol. 2016;152(6):670-5. DOI:10.1001/jamadermatol.2016.0091
11. Ji-Xu A, Lei DK, Maloney NJ, Worswick S. Clinical course, diagnostic patterns, and treatment outcomes in patients with pityriasis rubra pilaris. J Am Acad Dermatol.
2022;87(6):1450-1. DOI:10.1016/j.jaad.2022.07.039
12. Ghatnekar S, Shah N, Min MS, et al. Clinical features and eosinophilia in pityriasis rubra pilaris: A multicenter cohort. J Am Acad Dermatol. 2022;86:907-9. DOI:10.1016/j.jaad.2021.03.043
13. Yang CC, Shih IH, Lin WL, et al. Juvenile pityriasis rubra pilaris: Report of 28 cases in Taiwan. J Am Acad Dermatol. 2008;59(6):943-8. DOI:10.1016/j.jaad.2008.07.054
14. Allison DS, El-Azhary RA, Calobrisi SD, Dicken CH. Pityriasis rubra pilaris in children. J Am Acad Dermatol. 2002;47(3):386-9. DOI:10.1067/mjd.2002.124619
15. Blauvelt A, Nahass GT, Pardo RJ, Kerdel FA. Pityriasis rubra pilaris and HIV infection. J Am Acad Dermatol. 1991;24 24(5 Pt 1):703-5. DOI:10.1016/0190-9622(91)70106-c
16. Miralles ES, Núñez M, De Las Heras ME, et al. Pityriasis rubra pilaris and human immunodefciency virus infection. Br J Dermatol. 1995; 133(6):990-3.
DOI:10.1111/j.1365-2133.1995.tb06939.x
17. Nair PA, Sheth N. Atypical adult-onset pityriasis rubra pilaris in an HIV-positive adult male. Indian J Dermatol. 2018;63(6):522-4. DOI:10.4103/ijd.IJD_141_18
18. Kranyak A, Shuler M. Pityriasis rubra pilaris rapidly cleared with Ixekizumab in an HIV-positive patient. JAAD Case Rep. 2022;27:55-7. DOI:10.1016/j.jdcr.2022.07.015
19. Williams A, George A, Thomas EA, Koshy JM. Pityriasis rubra pilaris type 6: A case report in an AIDS patient. Indian J Sex Transm Dis AIDS. 2020;41(1):100-1. DOI:10.4103/ijstd.IJSTD_120_15
20. Allegue F, Fachal C, Iglesias B, Zulaica A. Facial discoid dermatosis: a new variant of pityriasis rubra pilaris? Actas Dermosifiliogr. 2022;113(7):728-31. DOI:10.1016/j.ad.2021.01.006
21. Li N, Tao J, Zhang J, et al. A novel mutation in a CARD14-associated papulosquamous eruption. Pediatr Dermatol. 2023;40(4):706-9. DOI:10.1111/pde.15254
22. Takeichi T, Terawaki S, Kubota Y, et al. A patient with CARD14-associated papulosquamous eruptions showing atopic dermatitis-like features. J Eur Acad Dermatol Venereol. 2021;35(1):e58-9. DOI:10.1111/jdv.16799
23. Ko CJ, Heald P, Antaya RJ, Bolognia JL. Facial discoid dermatosis. Int J Dermatol. 2010;49(2):189-92. DOI:10.1111/j.1365-4632.2009.04206.x
24. Salman A, Tekin B, Berenjian A, et al. Facial discoid dermatosis: A further case of a novel entity. J Dermatol. 2015;42(11):1132-3. DOI:10.1111/1346-8138.13045
25. Gan EY, Ng SK, Goh CL, Lee SSJ. Recalcitrant psoriasiform dermatosis of the face: is it related to pityriasis rubra pilaris? J Cutan Pathol. 2018;45(7):491-7. DOI:10.1111/cup.13148
26. Rypka KJ, Fulk TS, Afsaneh A, et al. Improvement of facial discoid dermatosis with ustekinumab treatment. JAMA Dermatol. 2022;158(9):1079-80. DOI:10.1001/jamadermatol.2022.2478
27. Davis AE, Raine BE, Swartzman I, et al. Rethinking pityriasis rubra pilaris as a paraneoplastic syndrome: two cases of pityriasis rubra pilaris with concomitant underlying malignancy. JAAD Case Rep. 2023;32:90-5. DOI:10.1016/j.jdcr.2022.12.007
28. Roenneberg S, Biedermann T. Pityriasis rubra pilaris: algorithms for diagnosis and treatment. J Eur Acad Dermatol Venereol. 2018;32(6):889-98. DOI:10.1111/jdv.14761
29. Naidoo A, Sibbald C, Fleming PJ, Piguet V. Use of biologics in pityriasis rubra pilaris refractory to first-line systemic therapy: A systematic review. J Cutan Med Surg. 2020;24(1):73-8. DOI:10.1177/1203475419887731
30. Kromer C, Sabat R, Celis D, Mössner R. Systemic therapies of pityriasis rubra pilaris: A systematic review. J Dtsch Dermatol Ges. 2019;17(3):243-59. DOI:10.1111/ddg.13718
31. Wassef C, Lombardi A, Rao BK. Adalimumab for the treatment of pityriasis rubra pilaris: A case report. Cutis. 2012;90(5):244-7.
32. Wohlrab J, Kreft B. Treatment of pityriasis rubra pilaris with ustekinumab. Br J Dermatol 2010;163(3):655-6. DOI:10.1111/j.1365-2133.2010.09855.x
33. Napolitano M, Lembo L, Fania L, et al. Ustekinumab treatment of pityriasis rubra pilaris: A report of five cases. J Dermatol. 2018;45(2):202-6. DOI:10.1111/1346-8138.14114
34. Haynes D, Strunck JL, Topham CA, et al. Evaluation of ixekizumab treatment for patients with pityriasis rubra pilaris: A single-arm trial. JAMA Dermatol. 2020;156(6):668-75. DOI:10.1001/jamadermatol.2020.0932
35. Boudreaux BW, Pincelli TP, Bhullar PK, et al. Secukinumab for the treatment of adult-onset pityriasis rubra pilaris: A single-arm clinical trial with transcriptomic analysis. Br J Dermatol. 2022;187(5):650-8. DOI:10.1111/bjd.21708
36. Ji-Xu A, Lei DK, Worswick S, et al. Patient and disease characteristics associated with psychiatric symptoms and impaired quality of life in pityriasis rubra pilaris. Br J Dermatol. 2022;187(6):1024-6. DOI:10.1111/bjd.21792
Авторы
В.П. Адаскевич*
УО «Витебский государственный ордена Дружбы народов медицинский университет», Витебск, Республика Беларусь
*vitebsk.derma@mail.ru