American Journal of Clinical Dermatology

, Volume 13, Issue 4, pp 247–260 | Cite as

Cicatricial (Scarring) Alopecias

An Overview of Pathogenesis, Classification, Diagnosis, and Treatment
Review Article


Cicatricial (scarring) alopecia forms a group of disorders in which the common final pathway is the destruction of the hair follicle unit that is replaced by fibrous tissue. Hair loss may occur as a primary event when the follicle is the main target of the disease process (primary cicatricial alopecias) or as a secondary event when the follicle act as an ‘innocent bystander’ in the course of a disease occurring outside of the follicular unit (secondary cicatricial alopecias). Permanent hair loss may also occur in the late phases of some nonscarring alopecias that are called ‘biphasic alopecias.’ Primary cicatricial alopecia accounts for 5% of all trichologic consultations at the Section of Dermatology, University of Genoa, Genoa, Italy. Considering that hair loss has a strong impact on patients’ psychology and quality of life, and that cicatricial alopecias can be associated with underlying systemic implications, it is extremely important that every clinician is familiar with the diagnosis and treatment of the different types of cicatricial alopecia.

An accurate clinical assessment integrated with (video) dermatoscopy and histopathologic studies permits a high standard performance of correct diagnoses. A brief review of our current knowledge of disease pathogenesis and the hypothetical disease mechanisms is presented. Some practical considerations for improving the 2001 North American Hair Research Society working classification of the primary cicatricial alopecias are suggested. The aim of treatment is to slow or stop the progression of the inflammatory waves and the scarring process at the earliest phase of involvement. Recommendations for therapy are based upon a literature review, personal experience, expected adverse effects, and some pragmatic considerations such as the cost and patient compliance.


  1. 1.
    Ross EK, Tan E, Shapiro J. Update on primary cicatricial alopecias. J Am Acad Dermatol 2005; 53: 1–37PubMedCrossRefGoogle Scholar
  2. 2.
    Whiting DA. Cicatricial alopecia: clinico-pathological findings and treatment. Clin Dermatol 2001; 19: 211–25PubMedCrossRefGoogle Scholar
  3. 3.
    Somani N, Bergfeld WF. Cicatricial alopecia: classification and histopathology. Dermatol Ther 2008; 21: 221–37PubMedCrossRefGoogle Scholar
  4. 4.
    Sperling LC, Cowper SE. The histopathology of primary cicatricial alopecia. Semin Cutan Med Surg 2006 Mar; 25: 41–50PubMedCrossRefGoogle Scholar
  5. 5.
    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 Mar; 160: 482–501PubMedCrossRefGoogle Scholar
  6. 6.
    Sellheyer K, Bergfeld WF. Histopathologic evaluation of alopecias. Am J Dermatopathol 2006; 28: 236–59PubMedCrossRefGoogle Scholar
  7. 7.
    McElwee KJ. Etiology of cicatricial alopecias: a basic science point of view. Dermatol Ther 2008; 21: 212–20PubMedCrossRefGoogle Scholar
  8. 8.
    McElwee KJ, Yu M, Park SW, et al. What can we learn from animal models of alopecia areata? Dermatology 2005; 211: 47–53PubMedCrossRefGoogle Scholar
  9. 9.
    Tobin DJ. Characterization of hair follicle antigens targeted by the anti-hair follicle immune response. J Investig Dermatol Symp Proc 2003; 8: 176–81PubMedCrossRefGoogle Scholar
  10. 10.
    Meyer KC, Klatte JE, Dinh HV, et al. Evidence that the bulge region is a site of relative immune privilege in human hair follicles. Br J Dermatol 2008; 159: 1077–85PubMedGoogle Scholar
  11. 11.
    Harries MJ, Meyer KC, Chaudhry IH, et al. Does collapse of immune privilege in the hair-follicle bulge play a role in the pathogenesis of primary cicatricial alopecia? Clin Exp Dermatol 2010; 35: 637–44PubMedCrossRefGoogle Scholar
  12. 12.
    Moresi JM, Horn TD. Distribution of Langerhans cells in human hair follicle. J Cutan Pathol 1997; 24: 636–40PubMedCrossRefGoogle Scholar
  13. 13.
    Hutchens KA, Balfour EM, Smoller BR. Comparison between Langerhans cell concentration in lichen planopilaris and traction alopecia with possible immunologic implications. Am J Dermatopathol 2011; 33: 277–80PubMedCrossRefGoogle Scholar
  14. 14.
    Pozdnyakova O, Mahalingam M. Involvement of the bulge region in primary scarring alopecia. J Cutan Pathol 2008; 35: 922–5PubMedCrossRefGoogle Scholar
  15. 15.
    Ito M, Liu Y, Yang Z, et al. Stem cells in the hair follicle bulge contribute to wound repair but not to homeostasis of the epidermis. Nat Med 2005; 11: 1351–4PubMedCrossRefGoogle Scholar
  16. 16.
    Hoang MP, Keady M, Mahalingam M. Stem cell markers (cytokeratin 15, CD34 and nestin) in primary scarring and nonscarring alopecia. Br J Dermatol 2009; 160: 609–15PubMedCrossRefGoogle Scholar
  17. 17.
    Karnik P, Tekeste Z, McCormick TS, et al. Hair follicle stem cell-specific PPARgamma deletion causes scarring alopecia. J Invest Dermatol 2009; 129: 1243–57PubMedCrossRefGoogle Scholar
  18. 18.
    Harries MJ, Paus R. Scarring alopecia and the PPAR-gamma connection. J Invest Dermatol 2009; 129: 1066–70PubMedCrossRefGoogle Scholar
  19. 19.
    Mirmirani P, Karnik P. Lichen planopilaris treated with a peroxisome proliferator-activated receptor gamma agonist. Arch Dermatol 2009; 145: 1363–6PubMedCrossRefGoogle Scholar
  20. 20.
    Harries MJ, Paus R. The pathogenesis of primary cicatricial alopecias. Am J Pathol 2010; 177: 2152–62PubMedCrossRefGoogle Scholar
  21. 21.
    Sundberg JP, Boggess D, Sundberg BA, et al. Asebia-2J (Scd1) (ab2J): a new allele and a model for scarring alopecia. Am J Pathol 2000; 156: 2067–75PubMedCrossRefGoogle Scholar
  22. 22.
    Rosenblum MD, Yancey KB, Olasz EB, et al. CD200, a “no danger” signal for hair follicles. Dermatol Sci 2006; 41: 165–74CrossRefGoogle Scholar
  23. 23.
    Sundberg JP, King Jr LE. Mouse models for the study of human hair loss. Dermatol Clin 1996; 14: 619–32PubMedGoogle Scholar
  24. 24.
    Rothnagel JA, Longley MA, Holder RA, et al. Genetic disorders of keratin: are scarring alopecias a sub-set? J Dermatol Sci 1994; 7 Suppl.: S164–9PubMedCrossRefGoogle Scholar
  25. 25.
    Romine KA, Rothschild JG, Hansen RC. Cicatricial alopecia and keratosis pilaris: keratosis follicularis spinulosa decalvans. Arch Dermatol 1997; 133: 381, 384PubMedCrossRefGoogle Scholar
  26. 26.
    Olsen EA, Bergfeld WF, Cotsarelis G, et al. Workshop on cicatricial alopecia: summary of North American Hair Research Society sponsored workshop on cicatricial alopecia. J Am Acad Dermatol 2003; 48: 103–10PubMedCrossRefGoogle Scholar
  27. 27.
    Rongioletti F, De Lucchi S, Meyes D, et al. Follicular mucinosis: a clinicopathologic, histochemical, immunohistochemical and molecular study comparing the primary benign form and the mycosis fungoides associated follicular mucinosis. J Cutan Pathol 2010; 37: 15–9PubMedCrossRefGoogle Scholar
  28. 28.
    Kossard S, Collins A, McCrossin I. Necrotizing lymphocytic folliculitis: the early lesion of acne necrotica (varioliformis). J Am Acad Dermatol 1987; 16: 1007–14PubMedCrossRefGoogle Scholar
  29. 29.
    Rongioletti F, Delmonte S, Rossi ME, et al. Erosive pustular dermatosis following cryotherapy and topical tretinoin for actinic keratoses. Clin Exp Dermatol 1999; 24: 499–500PubMedCrossRefGoogle Scholar
  30. 30.
    Fabbri P, Amato L, Chiarini C, et al. Scarring alopecia in discoid lupus erythematosus: a clinical, histopathologic and immunopathologic study. Lupus 2004; 13: 455–62PubMedCrossRefGoogle Scholar
  31. 31.
    Lopez-Tintos BO, Garcia-Hidalgo L, Orozco-Topete R. Dermoscopy in active discoid lupus. Arch Dermatol 2009; 145: 358PubMedCrossRefGoogle Scholar
  32. 32.
    Hordinsky M. Cicatricial alopecia: discoid lupus erythematosus. Dermatol Ther 2008; 21: 245–8PubMedCrossRefGoogle Scholar
  33. 33.
    Kang H, Alzolibani AA, Otberg N, et al. Lichen planopilaris. Dermatol Ther 2008; 21: 249–56PubMedCrossRefGoogle Scholar
  34. 34.
    Tan KT, Messenger AG. Frontal fibrosing alopecia: clinical presentations and prognosis. Br J Dermatol 2009; 160: 75–9PubMedCrossRefGoogle Scholar
  35. 35.
    Moreno-Ramírez D, Camacho Martínez F. Frontal fibrosing alopecia: a survey in 16 patients. J Eur Acad Dermatol Venereol 2005; 19: 700–5PubMedCrossRefGoogle Scholar
  36. 36.
    Rubegni P, Mandato F, Fimiani M. Frontal fibrosing alopecia: role of dermoscopy in differential diagnosis. Case Rep Dermatol 2010; 2: 40–5PubMedCrossRefGoogle Scholar
  37. 37.
    Poblet E, Jiménez F, Pascual A, et al. Frontal fibrosing alopecia versus lichen planopilaris: a clinicopathological study. Int J Dermatol 2006; 45: 375–80PubMedCrossRefGoogle Scholar
  38. 38.
    Tosti A, Piraccini BM, Iorizzo M, et al. Frontal fibrosing alopecia in post-menopausal women. J Am Acad Dermatol 2005; 52: 55–60PubMedCrossRefGoogle Scholar
  39. 39.
    Viglizzo G, Verrini AM, Rongioletti F. Familial Lassueur-Graham-Little-Piccardi syndrome. Dermatology 2004; 208: 142–4PubMedCrossRefGoogle Scholar
  40. 40.
    Whiting DA, Olsen EA. Central centrifugal cicatricial alopecia. Dermatol Ther 2008; 21: 268–78PubMedCrossRefGoogle Scholar
  41. 41.
    Alzolibani AA, Kang H, Otberg N, et al. Pseudopelade of Brocq. Dermatol Ther 2008; 21: 257–63PubMedCrossRefGoogle Scholar
  42. 42.
    Yu M, Bell RH, Ross EK, etal. Lichen planopilaris and pseudopelade of Brocq involve distinct disease associated gene expression patterns by microarray. J Dermatol Sci 2010; 57: 27–36PubMedCrossRefGoogle Scholar
  43. 43.
    Mirmirani P, Willey A, Headington JT, et al. Primary cicatricial alopecia: histopathologic findings do not distinguish clinical variants. J Am Acad Dermatol 2005; 52: 637–43PubMedCrossRefGoogle Scholar
  44. 44.
    Otberg N, Kang H, Alzolibani A, et al. Folliculitis decalvans. Dermatol Ther 2008; 21: 238–44PubMedCrossRefGoogle Scholar
  45. 45.
    Stefanato C. Histopathology of alopecia: a clinicopathological approach to diagnosis. Histopathology 2010; 54: 24–38CrossRefGoogle Scholar
  46. 46.
    Scheinfeld NS. A case of dissecting cellulitis and a review of the literature. Dermatol Online J 2003; 9 (1): 8PubMedGoogle Scholar
  47. 47.
    Kierland RR. Unusual pyodermas (hidrosadenitis suppurativa, acne conglobata, dissecting cellulitis of the scalp). Min Med 1951; 34: 319–25Google Scholar
  48. 48.
    Finner AM, Otberg N, Shapiro J. Secondary cicatricial and other permanent alopecias. Dermatol Ther 2008; 21: 279–94PubMedCrossRefGoogle Scholar
  49. 49.
    Frieden IJ. Aplasia cutis congenita: a clinical review and proposal for classification. J Am Acad Dermatol 1986; 14: 646–60PubMedCrossRefGoogle Scholar
  50. 50.
    Cribier B, Scrivener Y, Grosshans E. Tumors arising in nevus sebaceus: a study of 596 cases. J Am Acad Dermatol 2000; 42: 263–8PubMedCrossRefGoogle Scholar
  51. 51.
    Kishimoto H, Kishimoto H, Nagata S, et al. Rudimentary meningocele presenting with an annular alopecia. Eur J Dermatol 2000; 10: 215–6PubMedGoogle Scholar
  52. 52.
    Silva CY, Lenzy YM, Goldberg LJ. Temporal triangular alopecia with decreased follicular density. J Cutan Pathol 2010; 37: 597–9PubMedCrossRefGoogle Scholar
  53. 53.
    Wright AL, Messenger AG. Scarring alopecia in psoriasis. Acta Derm Venereol 1990; 70: 156–9PubMedGoogle Scholar
  54. 54.
    Schon MP, Reifenberger J, Gantke B, et al. Progressive cicatricial psoriatic alopecia in AIDS [in German]. Hautarzt 2000; 51: 935–8PubMedCrossRefGoogle Scholar
  55. 55.
    Martín JM, Pinazo I, Molina I, et al. Cicatricial pemphigoid of the Brunsting-Perry type. Int J Dermatol 2009; 48: 293–4PubMedCrossRefGoogle Scholar
  56. 56.
    Katta R, Nelson B, Chen D, et al. Sarcoidosis of the scalp: a case series and review of the literature. J Am Acad Dermatol 2000; 42: 690–2PubMedGoogle Scholar
  57. 57.
    Duque-Estrada B, Vincenzi C, Misciali C, et al. Alopecia secondary to mesotherapy. J Am Acad Dermatol 2009; 61: 707–9PubMedCrossRefGoogle Scholar
  58. 58.
    Cohen PR. Primary alopecia neoplastica versus secondary alopecia neoplastica: a new classification for neoplasm-associated scalp hair loss. J Cutan Pathol 2009; 36: 917–8PubMedCrossRefGoogle Scholar
  59. 59.
    Tran D, Sinclair RD, Schwarer AP, et al. Permanent alopecia following chemotherapy and bone marrow transplantation. Australas J Dermatol 2000; 41: 106–8PubMedCrossRefGoogle Scholar
  60. 60.
    Tosti A, Piraccini BM, Vincenzi C, et al. Permanent alopecia after busulfan chemotherapy. Br J Dermatol 2005 May; 152: 1056–8PubMedCrossRefGoogle Scholar
  61. 61.
    Scott DA. Disorders of the hair and scalp in blacks. Dermatol Clin 1988; 6: 387–95PubMedGoogle Scholar
  62. 62.
    Tosti A, Duque-Estrada B. Treatment strategies for alopecia. Expert Opin Pharmacother 2009; 10: 1017–26PubMedCrossRefGoogle Scholar
  63. 63.
    Harries MJ, Sinclair RD, Macdonald-Hull S, et al. Management of primary cicatricial alopecias: options for treatment. Br J Dermatol 2008; 159: 1–22PubMedCrossRefGoogle Scholar
  64. 64.
    Otberg N, Wu WY, McElwee KJ, et al. Diagnosis and management of primary cicatricial alopecia: part I. Skinmed 2008; 7: 19–26PubMedCrossRefGoogle Scholar
  65. 65.
    Assouly P, Reygagne P. Lichen planopilaris: update on diagnosis and treatment. Sem Cutan Med Surg 2009; 28: 3–10CrossRefGoogle Scholar
  66. 66.
    Donovan JC, Samrao A, Ruben BS, et al. Eyebrow regrowth in patients with frontal fibrosing alopecia treated with intralesional triamcinolone acetonide. Br J Dermatol 2010; 163: 1142–4PubMedCrossRefGoogle Scholar
  67. 67.
    Jouanique C, Reygagne P, Bachelez H, et al. Thalidomide is ineffective in the treatment of lichen planopilaris. J Am Acad Dermatol 2004; 51: 480–1PubMedCrossRefGoogle Scholar
  68. 68.
    Powell JJ, Dawber RP, Gatter K. Folliculitis decalvans including tufted folliculitis: clinical, histological and therapeutic findings. Br J Dermatol 1999; 140: 328–33PubMedCrossRefGoogle Scholar
  69. 69.
    Rose P, Shapiro R. Transplanting into scar tissue and areas of cicatricial alopecia. In: Unger WP, Shapiro R, editors. Hair transplantation. 4th ed. New York (NY): Marcel Dekker, 2004: 606–9Google Scholar
  70. 70.
    Kwon OS, Kim MH, Park SH, et al. Staged hair transplantation in cicatricial alopecia after carbon dioxide laser-assisted scar tissue remodeling. Arch Dermatol 2007; 143: 457–6PubMedCrossRefGoogle Scholar
  71. 71.
    Unger W, Unger R, Wesley C. The surgical treatment of cicatricial alopecia. Dermatol Ther. 2008; 21: 295–311PubMedCrossRefGoogle Scholar
  72. 72.
    Ross EK, Shapiro J. Management of hair loss. Dermatol Clin 2005; 23: 227–43PubMedGoogle Scholar

Copyright information

© Springer International Publishing AG 2012

Authors and Affiliations

  1. 1.Section of Dermatology, DissalUniversity of GenoaGenovaItaly

Personalised recommendations