Abstract
Scarring alopecias are a heterogeneous group of disorders characterized by the irreversible destruction of hair follicles. Follicular damage in scarring alopecias may be either primary or secondary. In primary cicatricial alopecia (PCA), the hair follicle itself is the principal target for destruction. Secondary cicatricial alopecias result from events not directed to the follicular unit that end up having an effect on and eventually destroying the follicle. Clinically, the hallmark of cicatricial alopecias is the loss of follicular openings, while under pathology, they are characterized by the replacement of follicular structures with fibrous tissue. Treatment of PCA is many times challenging, and there are no fully satisfactory evidence-based regimens available for the treatment of such conditions. Here, we will focus on the treatment of the main causes of PCA according to published data and our experience on the field. In scarring conditions, the central aim of treatment will not be hair regrowth but rather to reduce symptoms and, most importantly, to stop disease progression. Early treatment is key to minimizing the extent of permanent alopecia.
We will review the practical management of lichen planopilaris, frontal fibrosing alopecia, fibrosing alopecia in a pattern distribution, discoid lupus erythematosus, central centrifugal cicatricial alopecia, dissecting cellulitis, folliculitis decalvans, and acne keloidalis nuchae.
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Chiang YZ, Bundy C, Griffiths CEM, Paus R, Harries MJ. The role of beliefs: lessons from a pilot study on illness perception, psychological distress and quality of life in patients with primary cicatricial alopecia. Br J Dermatol. 2015;172(1):130–7. https://doi.org/10.1111/bjd.13259.
Whiting DA. Cicatricial alopecia: clinico-pathological findings and treatment. Clin Dermatol. 2001;19(2):211–25. http://www.ncbi.nlm.nih.gov/pubmed/11397600. Accessed October 10, 2016
Harries MJ, Trueb RM, Tosti A, et al. How not to get scar(r)ed: pointers to the correct diagnosis in patients with suspected primary cicatricial alopecia. Br J Dermatol. 2009;160(3):482–501. https://doi.org/10.1111/j.1365-2133.2008.09008.x.
Olsen EA, Bergfeld WF, Cotsarelis G, et al. Summary of North American Hair Research Society (NAHRS) - Sponsored workshop on cicatricial alopecia, Duke University Medical Center, February 10 and 11, 2001. J Am Acad Dermatol. 2003;48(1):103–10. https://doi.org/10.1067/mjd.2003.68.
Vaño-Galván S, Saceda-Corralo D, Blume-Peytavi U, et al. Frequency of the types of alopecia at 22 specialist hair clinics: a multicenter study. Ski Appendage Disord. 2019; In press.
Soares VC, Mulinari-Brenner F. Souza TE de. Lichen planopilaris epidemiology: a retrospective study of 80 cases. An Bras Dermatol. 2015;90(5):666–70. https://doi.org/10.1590/abd1806-4841.20153923.
Karnik P, Tekeste Z, McCormick TS, et al. Hair follicle stem cell-specific PPARγ deletion causes scarring alopecia. J Invest Dermatol. 2009;129(5):1243–57. https://doi.org/10.1038/jid.2008.369.Hair.
Imanishi H, Ansell DM, Chéret J, et al. Epithelial-mesenchymal stem cell transition in a human organ: lessons from lichen planopilaris. J Invest Dermatol. 2018;138:511. https://doi.org/10.1016/j.jid.2017.09.047.
Meinhard J, Stroux A, Lünnemann L, et al. Lichen planopilaris: epidemiology and prevalence of subtypes-a retrospective analysis in 104 patients. J Dtsch Dermatol Ges. 2014;12(3):229–35. https://doi.org/10.1111/ddg.12264.
Chiang C, Sah D, Cho BK, Ochoa BE, Price VH. Hydroxychloroquine and lichen planopilaris: efficacy and introduction of Lichen Planopilaris Activity Index scoring system. J Am Acad Dermatol. 2010;62(3):387–92. https://doi.org/10.1016/j.jaad.2009.08.054.
Rácz E, Gho C, Moorman P, Noordhoek Hegt V, Neumann H. Treatment of frontal fibrosing alopecia and lichen planopilaris: a systematic review. J Eur Acad Dermatol Venereol. 2013;27(12):1461–70. https://doi.org/10.1111/jdv.12139.
Cho BK, Sah D, Chwalek J, et al. Efficacy and safety of mycophenolate mofetil for lichen planopilaris. J Am Acad Dermatol. 2010;62(3):393–7. https://doi.org/10.1016/j.jaad.2009.05.018.
Mirmirani P, Willey A, Price VH. Short course of oral cyclosporine in lichen planopilaris. J Am Acad Dermatol. 2003;49(4):667–71. https://doi.org/10.1067/S0190-9622(03)00873-9.
Vañó-Galván S, Molina-Ruiz AM, Serrano-Falcón C, et al. Frontal fibrosing alopecia: a multicenter review of 355 patients. J Am Acad Dermatol. 2014;70(4):670–8. https://doi.org/10.1016/j.jaad.2013.12.003.
Moreno-Arrones OM, Saceda-Corralo D, Fonda-Pascual P, et al. Frontal fibrosing alopecia: clinical and prognostic classification. J Eur Acad Dermatol Venereol. 2017;31(10):1739–45. https://doi.org/10.1111/jdv.14287.
López-Pestaña A, Tuneu A, Lobo C, et al. Facial lesions in frontal fibrosing alopecia (FFA): clinicopathological features in a series of 12 cases. J Am Acad Dermatol. 2015;73(6):987.e1–6. https://doi.org/10.1016/j.jaad.2015.08.020.
Pirmez R, Duque-Estrada B, Donati A, et al. Clinical and dermoscopic features of lichen planus pigmentosus in 37 patients with frontal fibrosing alopecia. Br J Dermatol. 2016;175(6):1387–90. https://doi.org/10.1111/bjd.14722.
Donati A, Molina L, Doche I, et al. Facial papules in frontal fibrosing alopecia: evidence of vellus follicle involvement. Arch Dermatol. 2011;147:1424–7.
Pirmez R, Barreto T, Duque-Estrada B, Quintella DC, Cuzzi T. Facial papules in frontal fibrosing alopecia: beyond vellus hair follicle involvement. Ski Appendage Disord. 2018;4(3):145–9. https://doi.org/10.1159/000481695.
Heppt MV, Letulé V, Laniauskaite I, et al. Frontal fibrosing alopecia: a retrospective analysis of 72 patients from a German academic center. Facial Plast Surg. 2018;34(1):88–94. https://doi.org/10.1055/s-0037-1615281.
Murad A, Bergfeld W. 5-alpha-reductase inhibitor treatment for frontal fibrosing alopecia: an evidence-based treatment update. J Eur Acad Dermatol Venereol. 2018;32(8):1385–90. https://doi.org/10.1111/jdv.14930.
Rakowska A, Gradzińska A, Olszewska M, Rudnicka L. Efficacy of isotretinoin and acitretin in treatment of frontal fibrosing alopecia: retrospective analysis of 54 cases. J Drugs Dermatol. 2017;16(10):988–92.
Samrao A, Chew A-L, Price V. Frontal fibrosing alopecia: a clinical review of 36 patients. Br J Dermatol. 2010;163(6):1296–300. https://doi.org/10.1111/j.1365-2133.2010.09965.x.
Martínez-Velasco M, Vázquez-Herrera N, Misciali C, et al. Frontal fibrosing alopecia severity index: a trichoscopic visual scale that correlates thickness of peripilar casts with severity of inflammatory changes at pathology. Ski Appendage Disord. 2018;4(4):277–80. https://doi.org/10.1159/000487158.
Saceda-Corralo D, Moreno-Arrones ÓM, Fonda-Pascual P, et al. Development and validation of the frontal fibrosing alopecia severity score. J Am Acad Dermatol. 2018;78(3):522–9. https://doi.org/10.1016/j.jaad.2017.09.034.
Pirmez R, Duque-Estrada B, Barreto T, Quintella DC, Cuzzi T. Successful treatment of facial papules in frontal fibrosing alopecia with oral isotretinoin. Ski Appendage Disord. 2017;3(2):111–3. https://doi.org/10.1159/000464334.
Zinkernagel MS, Trueb RM. Fibrosing alopecia in a pattern distribution: patterned lichen planopilaris or androgenetic alopecia with a lichenoid tissue reaction pattern? Arch Dermatol. 2000;136(2):205–11. http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&dopt=Citation&list_uids=10677097.
Mardones F, Hott K, Martinez MC. Clinical study of fibrosing alopecia in a pattern distribution in a Latin American population. Int J Dermatol. 2018;57(2):e12–4. https://doi.org/10.1111/ijd.13871.
Missio DM, Dias MFRG, Trüeb RM. Familial cicatricial alopecia: report of familial frontal fibrosing alopecia and fibrosing alopecia in a pattern distribution. Int J Trichol. 2017;9(3):130–4. https://doi.org/10.4103/ijt.ijt_59_17.
Chiu H-Y, Lin S-J. Fibrosing alopecia in a pattern distribution. J Eur Acad Dermatol Venereol. 2010;24(9):1113–4. https://doi.org/10.1111/j.1468-3083.2010.03580.x.
Callen JP. Chronic cutaneous lupus erythematosus: clinical, laboratory, therapeutic, and prognostic examination of 62 patients. Arch Dermatol. 1982;118(6):412–6. https://doi.org/10.1001/archderm.1982.01650180046015.
Grönhagen CM, Fored CM, Granath F, Nyberg F. Cutaneous lupus erythematosus and the association with systemic lupus erythematosus: a population-based cohort of 1088 patients in Sweden. Br J Dermatol. 2011;164(6):1335–41. https://doi.org/10.1111/j.1365-2133.2011.10272.x.
Duque-Estrada B, Estrada BD, Tamler C, Sodré CT, Barcaui CB, Pereira FBC. Dermoscopy patterns of cicatricial alopecia resulting from discoid lupus erythematosus and lichen planopilaris. An Bras Dermatol. 2010;85(2):179–83. http://www.ncbi.nlm.nih.gov/pubmed/20520933. Accessed 20 Jan 2016.
Ross EK, Tan E, Shapiro J. Update on primary cicatricial alopecias. J Am Acad Dermatol. 2005;53(1):1–37. https://doi.org/10.1016/j.jaad.2004.06.015.
Roenigk HJ, Martin J, Eichorn P, Gilliam J. Discoid lupus erythematosus. Diagnostic features and evaluation of topical corticosteroid therapy. Cutis. 1980;25:281–5.
Harries MJ, Sinclair RD, Macdonald-Hull S, Whiting DA, Griffiths CE, Paus R. Management of primary cicatricial alopecias: options for treatment. Br J Dermatol. 2008;159:1–22.
Kuhn A, Aberer E, Bata-Csörgő Z, et al. S2k guideline for treatment of cutaneous lupus erythematosus – guided by the European Dermatology Forum (EDF) in cooperation with the European Academy of Dermatology and Venereology (EADV). J Eur Acad Dermatol Venereol. 2017;31(3):389–404. https://doi.org/10.1111/jdv.14053.
Garza-Mayers A, McClurkin M, Smith G. Review of treatment for discoid lupus erythematosus. Dermatol Ther. 2016;29(4):274–83. https://doi.org/10.1111/dth.12358.
Gathers RC, Jankowski M, Eide M, Lim HW. Hair grooming practices and central centrifugal cicatricial alopecia. J Am Acad Dermatol. 2009;60(4):574–8. https://doi.org/10.1016/j.jaad.2008.10.064.
LoPresti P, Papa CM, Kligman AM. Hot comb alopecia. Arch Dermatol. 1968;98(3):234–8. http://www.ncbi.nlm.nih.gov/pubmed/5673883. Accessed 11 Oct 2018.
Miteva M, Tosti A. Central centrifugal cicatricial alopecia presenting with irregular patchy alopecia on the lateral and posterior scalp. Ski Appendage Disord. 2015;1(1):1–5. https://doi.org/10.1159/000370315.
Callender VD, Wright DR, Davis EC, Sperling LC. Hair breakage as a presenting sign of early or occult central centrifugal cicatricial alopecia: clinicopathologic findings in 9 patients. Arch Dermatol. 2012;148(9):1047–52. https://doi.org/10.1001/archdermatol.2011.3428.
Miteva M, Tosti A. Dermatoscopic features of central centrifugal cicatricial alopecia. J Am Acad Dermatol. 2014;71(3):443–9. https://doi.org/10.1016/j.jaad.2014.04.069.
Khumalo NP, Gumedze F. Traction: risk factor or coincidence in central centrifugal cicatricial alopecia? Br J Dermatol. 2012;167(5):1191–3. https://doi.org/10.1111/j.1365-2133.2012.11050.x.
Gathers RC, Lim HW. Central centrifugal cicatricial alopecia: past, present, and future. J Am Acad Dermatol. 2009;60(4):660–8. https://doi.org/10.1016/j.jaad.2008.09.066.
Price VH. The medical treatment of cicatricial alopecia. Semin Cutan Med Surg. 2006;25(1):56–9. https://doi.org/10.1016/j.sder.2006.01.008.
Spitzer L. Dermatitis follicularis et perifollicularis conglobate. Dermatol Zeitschrift. 1903;10:109–20.
Van der Zee HH, Laman JD, Boer J, Prens EP. Hidradenitis suppurativa: viewpoint on clinical phenotyping, pathogenesis and novel treatments. Exp Dermatol. 2012;21(10):735–9. https://doi.org/10.1111/j.1600-0625.2012.01552.x.
Hoffman E. Perifolliculitis capitis abscedens et suffodiens. Dermatol Zeitschrift. 1908;15:122–3.
Segurado-Miravalles G, Camacho-Martínez FM, Arias-Santiago S, et al. Epidemiology, clinical presentation and therapeutic approach in a multicentre series of dissecting cellulitis of the scalp. J Eur Acad Dermatol Venereol. 2016;31:e199–200. https://doi.org/10.1111/jdv.13948.
Tosti A, Torres F, Miteva M. Dermoscopy of early dissecting cellulitis of the scalp simulates alopecia areata. Actas Dermosifiliogr. 2013;104(1):92–3. https://doi.org/10.1016/j.ad.2012.05.008.
Segurado-Miravalles G, Camacho-Martinez F, Arias-Santiago S, et al. Trichoscopy of dissecting cellulitis of the scalp: exclamation mark hairs and white dots as markers of disease chronicity. J Am Acad Dermatol. 2016;75(6):1267–8. https://doi.org/10.1016/j.jaad.2016.08.035.
Jerome MA, Laub DR. Dissecting cellulitis of the scalp: case discussion, unique considerations, and treatment options. Eplasty. 2014;14:ic17. http://www.ncbi.nlm.nih.gov/pubmed/24966998. Accessed 15 May 2017
Otberg N, Kang H, Alzolibani AA, Shapiro J. Folliculitis decalvans. Dermatol Ther. 2008;21(4):238–44. https://doi.org/10.1111/j.1529-8019.2008.00204.x.
Vañõ-Galván S, Molina-Ruiz AM, Fernández-Crehuet P, et al. Folliculitis decalvans: a multicentre review of 82 patients. J Eur Acad Dermatol Venereol. 2015;29(9):1750–7. https://doi.org/10.1111/jdv.12993.
Ekmekci TR, Koslu A. Tufted hair folliculitis causing skullcap-pattern cicatricial alopecia [8]. J Eur Acad Dermatol Venereol. 2006;20(2):227–9. https://doi.org/10.1111/j.1468-3083.2005.01384.x.
Fernández-Crehuet P, Vañó-Galván S, Molina-Ruiz AM, et al. Trichoscopic features of folliculitis decalvans: results in 58 patients. Int J Trichol. 2017;9(3):140–1. https://doi.org/10.4103/ijt.ijt_85_16.
Rudnicka L, Olszewska M, Rakowska A, Slowinska M. Trichoscopy update 2011. J Dermatol Case Rep. 2011;5(4):82–8. https://doi.org/10.3315/jdcr.2011.1083.
Miguel-Gómez L, Rodrigues-Barata AR, Molina-Ruiz A, et al. Folliculitis decalvans: effectiveness of therapies and prognostic factors in a multicenter series of 60 patients with long-term follow-up. J Am Acad Dermatol. 2018;79:878–83. https://doi.org/10.1016/j.jaad.2018.05.1240.
Dinehart SM, Herzberg AJ, Kerns BJ, Pollack SV. Acne keloidalis: a review. J Dermatol Surg Oncol. 1989;15(6):642–7. https://doi.org/10.1111/j.1524-4725.1989.tb03603.x.
Adegbidi H, Atadokpede F, do Ango-Padonou F, Yedomon H. Keloid acne of the neck: epidemiological studies over 10 years. Int J Dermatol. 2005;44(SUPPL. 1):49–50. https://doi.org/10.1111/j.1365-4632.2005.02815.x.
Ogunbiyi A. Acne keloidalis nuchae: prevalence, impact, and management challenges. Clin Cosmet Investig Dermatol. 2016;9:483–9. https://doi.org/10.2147/CCID.S99225.
Maranda EL, Simmons BJ, Nguyen AH, Lim VM, Keri JE. Treatment of acne keloidalis nuchae: a systematic review of the literature. Dermatol Ther (Heidelb). 2016;6(3):363–78. https://doi.org/10.1007/s13555-016-0134-5.
Fernandez AP. Updated recommendations on the use of hydroxychloroquine in dermatologic practice. J Am Acad Dermatol. 2017;76(6):1176–82. https://doi.org/10.1016/j.jaad.2017.01.012.
Hughes G. Hydroxychloroquine: an update. Lupus. 2018;27(9):1402–3. https://doi.org/10.1177/0961203318787040.
Callen JP, Camisa S. Antimalarial agents. In: Wolverton SE, editor. Comprehensive dermatologic drug therapy. 3th ed. USA: Elsevier; 2013. p. 241–51.
Plaquinol™ drug information. http://www.anvisa.gov.br/datavisa/fila_bula/frmVisualizarBula.asp?pNuTransacao=7415002015&pIdAnexo=2812605. Accessed on 13 Feb 2019.
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Vañó-Galván, S., Saceda-Corralo, D., Pirmez, R. (2020). Scarring Alopecias. In: Tosti, A., Asz-Sigall, D., Pirmez, R. (eds) Hair and Scalp Treatments. Springer, Cham. https://doi.org/10.1007/978-3-030-21555-2_11
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