Vitiligo pp 53-71 | Cite as

Segmental Vitiligo

  • Seung-Kyung Hann
  • Hsin-Su Yu
  • Cheng-Che Eric Lan
  • Ching-Shuang Wu
  • Yvon Gauthier
  • Laïla Benzekri
  • Alain TaïebEmail author


Segmental vitiligo (SV) has been historically opposed to vitiligo/nonsegmental vitiligo (NSV). However, evidence that the two forms are not mutually exclusive (delineation of mixed vitiligo) and share similar inflammatory features has challenged classical views over the last decade. SV belongs to the clinical spectrum of vitiligo, including inflammatory skin features. SV has usually an early onset and spreads rapidly in the affected segment. The hair follicle melanocytic compartment is frequently involved. Distribution in SV parallels that of other acquired pigmentation disorders such as nevus spilus, pointing out to some underlying developmental defect. Neurogenic influences have been highlighted by striking clinical observations and experimental studies. Classification of SV is needed to establish a prognosis, and two recent classifications for cephalic and truncal SV have been proposed. Segmental vitiligo is a perfect model for studying repigmentation since it is a relative stable disease with limited active melanocyte loss. Early aggressive medical treatment of SV is currently recommended.


  1. 1.
    Koga M. Vitiligo: a new classification and therapy. Br J Dermatol. 1977;97:255–61.CrossRefGoogle Scholar
  2. 2.
    Klippel M, Weill HP. Radicular distribution of vitiligo and nevi. Presse Med. 1922;30:388–90.Google Scholar
  3. 3.
    Koga M, Tango T. Clinical features and course of type A and type B vitiligo. Br J Dermatol. 1988;118:223–8.CrossRefPubMedPubMedCentralGoogle Scholar
  4. 4.
    Musumeci V. Sulla distribuzione radicolare della vitiligine. Minerva Dermatol. 1951;26:77–9.PubMedPubMedCentralGoogle Scholar
  5. 5.
    Ortonne JP, Mosher DB, Fitzpatrick TB. Vitiligo and other hypomelanosis of hair and skin. New York, NY: Plenum Medical Book Company; 1983. p. 250–8.CrossRefGoogle Scholar
  6. 6.
    Scholtz JR, Williamson C. Vitiligo in apparent neural distribution. Arch Dermatol Syphil. 1951;64:366–8.CrossRefGoogle Scholar
  7. 7.
    Touraine A, Brizard A. La topographie radiculaire du vitiligo. Bull Soc Fr Dermotol Syphil. 1935;42:505–15.Google Scholar
  8. 8.
    Nelhaus G. Acquired unilateral vitiligo and poliosis of the head and subacute encephalitis with partial recovery. Neurology. 1970;20:965–74.CrossRefGoogle Scholar
  9. 9.
    Costea V. Leucoderma patches in the course of traumatic paralysis of the brachial plexus in a subject with insular cavities. Act Dermat Venerol. 1961;2:161–6.Google Scholar
  10. 10.
    Lerner AB. Vitiligo. J Invest Dermatol. 1959;39:285–310.CrossRefGoogle Scholar
  11. 11.
    Lerner AB. Sympathectomy and gray hair. Arch Dermatol. 1966a;93:235–6.CrossRefPubMedPubMedCentralGoogle Scholar
  12. 12.
    Lerner AB. Vitiligo and sympathectomy. Arch Dermatol. 1966b;94:269–78.CrossRefPubMedPubMedCentralGoogle Scholar
  13. 13.
    Ferriol L. Vitiligo et tumeurs neurologiques de la moelle. Rev Neurol Paris. 1905;13:282–6.Google Scholar
  14. 14.
    Tremiteria S. Vitiligo from spinal anesthesia. Rinazcenca medica. 1927;4:107–8.Google Scholar
  15. 15.
    Breatnach AS, Bors S, Willie L, et al. Electron microscopy of peripheral nerve terminals and marginal melanocytes in vitiligo. J Invest Dermatol. 1966;47:125–40.CrossRefGoogle Scholar
  16. 16.
    Gauthier Y, Surlève-Bazeille JE. Ultrastructure des fibres nerveuses périphériques dermiques dans le vitiligo. Bull Soc Fr Dermatol Syphil. 1974;81:550–4.Google Scholar
  17. 17.
    Al’Abadie MS, Senior HJ, et al. Neuropeptide and neuronal marker studies in vitiligo. Br J Dermatol. 1994;131:160–5.CrossRefPubMedPubMedCentralGoogle Scholar
  18. 18.
    Jacobowitz DM, Laties AM. Direct adrenergic innervation of a teleost melanophore. Anat Rec. 1968;162:501–4.CrossRefPubMedPubMedCentralGoogle Scholar
  19. 19.
    McGuire J. Adrenergic control of melanocytes. Arch Dermatol. 1970;101:173–80.CrossRefPubMedPubMedCentralGoogle Scholar
  20. 20.
    Fabian G. The spread of black pigment of the denervated skin of the guinea pig. Acta Biol Acad Sci Hung. 1951;4:471–9.Google Scholar
  21. 21.
    Lerner AB. In: Kawanamura T, et al., editors. Neural control of pigment cells: biology of normal and abnormal melanocytes. Tokyo: University Park Press; 1971. p. 3–16.Google Scholar
  22. 22.
    Chanco-Turner ML, Lerner AB. Physiologic changes in vitiligo. Arch Dermatol. 1965;91:390–6.CrossRefPubMedPubMedCentralGoogle Scholar
  23. 23.
    Gauthier Y, Surlève-Bazeille JE, Gauthier O. Bilan de l’activité cholinestérasique dans le vitiligo: son interêt physiopathologique. Bull Soc Fr Dermatol Syphil. 1976;81:321.Google Scholar
  24. 24.
    Iyengar B. Modulation of melanocyte activity by acetylcholine. Acta Anat. 1989;136:139–41.CrossRefPubMedPubMedCentralGoogle Scholar
  25. 25.
    Jacklin NH. Depigmentation of the eyelids in eserine allergy. Am J Ophtalmol. 1965;59:890–902.CrossRefGoogle Scholar
  26. 26.
    Schallreuter KU, Elwary SM, Gibbons NC, et al. Activation/desactivation of acetylcholinesterase by H2O2 more evidence for oxydative stress in vitiligo. Biochem Biophys Res Commun. 2004;315:502–8.CrossRefPubMedPubMedCentralGoogle Scholar
  27. 27.
    Bamshad J. COMT in Skin. J Invest Dermatol. 1964;43:111–3.CrossRefPubMedPubMedCentralGoogle Scholar
  28. 28.
    Bir LS, Aktan S. Sympathetic skin response in psoriasis and vitiligo. J Auton Nerv Syst. 1999;77(1):68–71.CrossRefPubMedPubMedCentralGoogle Scholar
  29. 29.
    Stajano C, Martirena H. Bilateral peripheral distribution of vitiligo according to nerve segments. Ac Fac Med Montevideo. 1926;11:563–81.Google Scholar
  30. 30.
    Touraine A, Picquart A. Maladie de Recklinghausen à pigmentation systématisée, vitiligo, pelade. Bull Soc F Dermtol Syphil. 1937;44:81–5.Google Scholar
  31. 31.
    Wu CS, Yu HS, Chang HR, et al. Cutaneous blood flow and adrenoceptor response increase in segmental-type vitiligo lesions. J Dermatol Sci. 2000;23:53–62.CrossRefGoogle Scholar
  32. 32.
    Morrone A, Picardo M, De Luca C. Catecholamines and vitiligo. Pigm Cell Res. 1992;5:65–9.CrossRefGoogle Scholar
  33. 33.
    Blaschko A. A “Die Nervenverteilung in der Haut in ihre Beziehung zu den Erkrankungen der Haut”. Beilage zu den Verhandlungen der Deutschen Dermatologischen Gesellschaft VII Congress, Breslau, 1901Google Scholar
  34. 34.
    Happle R. Lyonization and the lines of Blaschko. Hum Genet. 1985;70:200–6.CrossRefPubMedPubMedCentralGoogle Scholar
  35. 35.
    Paller AS, Syder AJ, Chan YM, et al. Genetic and clinical mosaicism in a type of epidermal nevus. N Engl J Med. 1994;331:1408–15.CrossRefPubMedPubMedCentralGoogle Scholar
  36. 36.
    Taibjee SM, Bennett DC, Moss C. Abnormal pigmentation in hypomelanosis of Ito and pigmentary mosaicism: the role of pigmentary genes. Br J Dermatol. 2004;151:269–82.CrossRefPubMedPubMedCentralGoogle Scholar
  37. 37.
    Morice-Picard F, Boralevi F, Lepreux S, et al. Severe linear form of granuloma annulare along Blaschko’s lines preceding the onset of a classical form of granuloma annulare in a child. Br J Dermatol. 2007;157:1056–8.CrossRefPubMedPubMedCentralGoogle Scholar
  38. 38.
    Taïeb A, Morice-Picard F, Jouary T, et al. Segmental vitiligo as the possible expression of cutaneous somatic mosaicism: implications for common non-segmental vitiligo. Pigment Cell Melanoma Res. 2008;21:646–52.CrossRefGoogle Scholar
  39. 39.
    Happle R. [Patterns on the skin. New aspects of their embryologic and genetic causes, German] Hautarzt. 2004;55:960–1, 964–8.Google Scholar
  40. 40.
    Taieb A. Intrinsic and extrinsic pathomechanisms in vitiligo. Pigment Cell Res. 2000;13:41–7.CrossRefGoogle Scholar
  41. 41.
    Nazzaro V, Ermacora E, Santucci B, Caputo R. Epidermolytic hyperkeratosis: generalized form in children from parents with systematized linear form. Br J Dermatol. 1990;122:417–22.CrossRefPubMedPubMedCentralGoogle Scholar
  42. 42.
    Moellmann G, McGuire J, Lerner AB. Ultrastructure and cell biology of pigment cells. Intracellular dynamics and the fine structure of melanocytes with special reference to the effects of MSH and cyclic AMP on microtubules and 10-nm filaments. Yale J Biol Med. 1973;46:337–60.PubMedPubMedCentralGoogle Scholar
  43. 43.
    Happle R, Assim A. The lines of Blaschko on the head and neck. J Am Acad Dermatol. 2001;44:612–5.CrossRefPubMedPubMedCentralGoogle Scholar
  44. 44.
    Bolognia JL, Orlow SJ, Glick SA. Lines of Blaschko. J Am Acad Dermatol. 1994;31:157–90.CrossRefPubMedPubMedCentralGoogle Scholar
  45. 45.
    Baba M, Akcali C, Seçkin D, Happle R. Segmental lentiginosis with ipsilateral nevus depigmentosus: another example of twin spotting? Eur J Dermatol. 2002;12:319–21.PubMedPubMedCentralGoogle Scholar
  46. 46.
    van Geel NA, Mollet IG, De Schepper S, Tjin EP, Vermaelen K, Clark RA, Kupper TS, Luiten RM, Lambert J. First histopathological and immunophenotypic analysis of early dynamic events in a patient with segmental vitiligo associated with halo nevi. Pigment Cell Melanoma Res. 2010;23:375–84.CrossRefGoogle Scholar
  47. 47.
    Shin J, Kang HY, Kim KH, Park CJ, Oh SH, Lee SC, Lee S, Choi GS, Hann SK. Involvement of T cells in early evolving segmental vitiligo. Clin Exp Dermatol. 2016;41:671–4.CrossRefGoogle Scholar
  48. 48.
    Attili VR, Attili SK. Segmental and generalized vitiligo: both forms demonstrate inflammatory histopathological features and clinical mosaicism. Indian J Dermatol. 2013;58:433–8.CrossRefPubMedPubMedCentralGoogle Scholar
  49. 49.
    Taïeb A, Picardo M, VETF Members. The definition and assessment of vitiligo: a consensus report of the Vitiligo European Task Force. Pigment Cell Res. 2007;20:27–35.CrossRefPubMedPubMedCentralGoogle Scholar
  50. 50.
    Hann SK, Lee HJ. Segmental vitiligo: clinical findings in 208 patients. J Am Acad Dermatol. 1996;35:671–4.CrossRefPubMedPubMedCentralGoogle Scholar
  51. 51.
    van Geel N, De Lille S, Vandenhaute S, Gauthier Y, Mollet I, Brochez L, Lambert J. Different phenotypes of segmental vitiligo based on a clinical observational study. J Eur Acad Dermatol Venereol. 2011;25:673–8.CrossRefGoogle Scholar
  52. 52.
    El Mofty AM, El Mofty M. Vitiligo: a symptom complex. Int J Dermatol. 1980;19:238–47.CrossRefGoogle Scholar
  53. 53.
    Park K, Youn JL, Lee YS. A clinical study of 326 cases of vitiligo. Korean J Dermatol. 1988;26:200–5.Google Scholar
  54. 54.
    Ezzedine K, Diallo A, Léauté-Labrèze C, Mossalayi D, Gauthier Y, Bouchtnei S, Cario-André M, Seneschal J, Boralevi F, Jouary T, Taieb A. Multivariate analysis of factors associated with early-onset segmental and nonsegmental vitiligo: a prospective observational study of 213 patients. Br J Dermatol. 2011;165:44–9.CrossRefPubMedPubMedCentralGoogle Scholar
  55. 55.
    Kim DY, Oh SH, Hann SK. Classification of segmental vitiligo on the face: clues for prognosis. Br J Dermatol. 2011;164:1004–9.CrossRefPubMedPubMedCentralGoogle Scholar
  56. 56.
    van Geel N, Bosma S, Boone B, Speeckaert R. Classification of segmental vitiligo on the trunk. Br J Dermatol. 2014;170:322–7.CrossRefPubMedPubMedCentralGoogle Scholar
  57. 57.
    Happle R. Mosaicism in human skin. Understanding the patterns and mechanisms. Arch Dermatol. 1993;129:1460–70.CrossRefGoogle Scholar
  58. 58.
    Lee HS, Hann SK. Bilateral segmental vitiligo. Ann Dermatol(Seoul). 1998;10:129–31.CrossRefGoogle Scholar
  59. 59.
    Howitz J, Brodthagen H, Schwartz M, et al. Prevalence of vitiligo. Epidemiological survey on the Isle of Bornholm, Denmark. Arch Dermatol. 1977;113:47–52.CrossRefGoogle Scholar
  60. 60.
    Yaar M, Gilchrest BA. Human melanocyte growth and differentiation: a decade of new data. J Invest Dermatol. 1991;97:611–7.CrossRefPubMedPubMedCentralGoogle Scholar
  61. 61.
    Staricco RG. Activation of the amelanotic melanocytes in the outer root sheath of the hair follicle following ultra violet rays exposure. J Invest Dermatol. 1962;39:163–4.CrossRefPubMedPubMedCentralGoogle Scholar
  62. 62.
    Cui J, Shen LY, Wang GC. Role of hair follicles in the repigmentation of vitiligo. J Invest Dermatol. 1991;97:410–6.CrossRefGoogle Scholar
  63. 63.
    Yu HS. Melanocyte destruction and repigmentation in vitiligo: a model for nerve cell damage and regrowth. J Biomed Sci. 2002;9:564–73.CrossRefPubMedPubMedCentralGoogle Scholar
  64. 64.
    Bartosik J, Wulf HC, Kobayasi T. Melanin and melanosome complexes in long standing stable vitiligo-an ultrastructural study. Eur J Dermatol. 1998;8:95–7.Google Scholar
  65. 65.
    Silverberg NB, Lin P, Travis L, et al. Tacrolimus ointment promotes repigmentation of vitiligo in children: a review of 57 cases. J Am Acad Dermatol. 2004;51:760–6.CrossRefPubMedPubMedCentralGoogle Scholar
  66. 66.
    Tobin DJ, Bystryn JC. Different populations of melanocytes are present in hair follicles and epidermis. Pigment Cell Res. 1996;9:304–10.CrossRefPubMedPubMedCentralGoogle Scholar
  67. 67.
    Tallab T, Joharji H, Bahamdan K, et al. Response of vitiligo to PUVA therapy in Saudi patients. Int J Dermatol. 2005;44:556–8.CrossRefPubMedPubMedCentralGoogle Scholar
  68. 68.
    Khalid M, Mutjaba G. Response of segmental vitiligo to 0.05% clobetasol propionate cream. Int J Dermatol. 1998;37:705–8.CrossRefPubMedPubMedCentralGoogle Scholar
  69. 69.
    Wu CS, Yu CL, Wu CS, et al. Narrow-band ultraviolet-B stimulates proliferation and migration of cultured melanocytes. Exp Dermatol. 2004;13:755–63.CrossRefGoogle Scholar
  70. 70.
    Kao CH, Yu HS. Comparison of the effect of 8-methoxypsoralen (8-MOP) plus UVA (PUVA) on human melanocytes in vitiligo vulgaris and in vitro. J Invest Dermatol. 1992;98:734–40.CrossRefPubMedPubMedCentralGoogle Scholar
  71. 71.
    Lei TC, Virador V, Yasumoto K, et al. Stimulation of melanoblast pigmentation by 8-methoxypsoralen: the involvement of microphthalmia-associated transcription factor, the protein kinase A signal pathway, and proteasome-mediated degradation. J Invest Dermatol. 2002;119:1341–9.CrossRefPubMedPubMedCentralGoogle Scholar
  72. 72.
    Westerhof W, Nieuweboer-Krobotova L. Treatment of vitiligo with UV-B radiation vs topical psoralen plus UV-A. Arch Dermatol. 1997;133:1525–8.CrossRefPubMedPubMedCentralGoogle Scholar
  73. 73.
    Ozawa M, Ferenczi K, Kikuchi T, et al. 312-nanometer ultraviolet B light (narrow-band UVB) induces apoptosis of T cells within psoriatic lesions. J Exp Med. 1999;189:711–8.CrossRefPubMedPubMedCentralGoogle Scholar
  74. 74.
    Anbar TS, Westerhof W, Abdel-Rahman AT, et al. Evaluation of the effects of NBUVB in both segmental and non-segmental vitiligo affecting different body sites. Photodermatol Photoimmunol Photomed. 2006;22:157–63.CrossRefPubMedPubMedCentralGoogle Scholar
  75. 75.
    Yu HS, Wu CS, Yu CL, et al. Helium Neon laser irradiation stimulates migration and proliferation in melanocytes and induces repigmentation in segmental type vitiligo. J Invest Dermatol. 2003;120:56–64.CrossRefPubMedPubMedCentralGoogle Scholar
  76. 76.
    Lan CC, Wu CS, Chiou MH, et al. Low-energy helium-neon laser induces locomotion of the immature melanoblasts and promotes melanogenesis of the more differentiated melanoblasts: recapitulation of vitiligo repigmentation in vitro. J Invest Dermatol. 2006;126:2119–26.CrossRefPubMedPubMedCentralGoogle Scholar
  77. 77.
    Lan CC, Chen GS, Chiou MH, et al. FK506 promotes melanocyte and melanoblast growth and creates a favourable milieu for cell migration via keratinocytes: possible mechanisms of how tacrolimus ointment induces repigmentation in patients with vitiligo. Br J Dermatol. 2005;153:498–505.CrossRefPubMedPubMedCentralGoogle Scholar
  78. 78.
    Kang HY, Choi YM. FK506 increases pigmentation and migration of human melanocytes. Br J Dermatol. 2006;155:1037–40.CrossRefPubMedPubMedCentralGoogle Scholar
  79. 79.
    Khullar SM, Brodin P, Barkvoll P, et al. Preliminary study of low-level laser for treatment of longstanding sensory alteration in the inferior alveolar nerve. J Oral Maxillofac Surg. 1996;54:2–7.CrossRefPubMedPubMedCentralGoogle Scholar
  80. 80.
    Rochkind S, Rousso M, Nissan M, et al. Systemic effects of low-power laser irradiation on the peripheral and central nervous system, cutaneous wounds, and burns. Lasers Surg Med. 1989;9:174–82.CrossRefPubMedPubMedCentralGoogle Scholar
  81. 81.
    Ständer S, Ständer H, Seeliger S, et al. Topical pimecrolimus and tacrolimus transiently induce neuropeptide release and mast cell degranulation in murine skin. Br J Dermatol. 2007;156:1020–6.CrossRefPubMedPubMedCentralGoogle Scholar
  82. 82.
    Kano Y, Nohno T, Hasegawa T, et al. Immunosuppressant FK506 induces sustained activation of MAP kinase and promotes neurite outgrowth in PC12 mutant cells incapable of differentiating. Cell Struct Func. 2002;27:393–8.CrossRefGoogle Scholar
  83. 83.
    Hann SK, Im S, Park YK, Hur W. Repigmentation of leukotrichia by epidermal grafting and systemic psoralen plus UV-A. Arch Dermatol. 1992;128:998–9.CrossRefGoogle Scholar
  84. 84.
    Pavlovic S, Daniltchenko M, Tobin DJ, et al. Further exploring the brain-skin connection: stress worsens dermatitis via substance P-dependent neurogenic inflammation in mice. J Invest Dermatol. 2008;128:434–46.CrossRefPubMedPubMedCentralGoogle Scholar
  85. 85.
    Nishimura EK, Jordan SA, Oshima H, et al. Dominant role of the niche in melanocyte stem-cell fate determination. Nature. 2002;416:854–60.CrossRefPubMedPubMedCentralGoogle Scholar
  86. 86.
    Yonetani S, Moriyama M, Nishigori C, et al. In vitro expansion of immature melanoblasts and their ability to repopulate melanocyte stem cells in the hair follicule. J Invest Dermatol. 2008;128:408–20.CrossRefGoogle Scholar

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© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Seung-Kyung Hann
    • 1
  • Hsin-Su Yu
    • 2
  • Cheng-Che Eric Lan
    • 2
  • Ching-Shuang Wu
    • 3
  • Yvon Gauthier
    • 4
  • Laïla Benzekri
    • 5
  • Alain Taïeb
    • 4
    Email author
  1. 1.Korea Institute of Vitiligo ResearchDrs. Woo & Hann’s skin clinicSeoulSouth Korea
  2. 2.Department of DermatologyKaohsiung Medical UniversityKaohsiungTaiwan
  3. 3.Faculty of Biomedical Laboratory ScienceKaohsiung Medical UniversityKaohsiungTaiwan
  4. 4.Service de DermatologieHôpital Saint-André, CHU de BordeauxBordeauxFrance
  5. 5.Department of DermatologyCHU Ibn SinaRabatMorocco

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