Vitiligo || Segmental Vitiligo

M. Picardo and A. Taïeb (eds.), Vitiligo, 41DOI 10.1007/978-3-540-69361-1_1.3.2, © Springer-Verlag Berlin Heidelberg 2010

Segmental Vitiligo

Seung-Kyung Hann, Yvon Gauthier, and Laila Benzekri

1.3.2

There is in general no problem in defi ning SV (Chap. 1.2.1). However, interpretations have varied concerning its relation to nonsegmental forms. Furthermore, there is still a debate about SV clinical subclassifi cations, mostly according to the interpre-tation of the relative importance of a presupposed dermatomal (sensitive nerve distribution) and/or associated developmental anomaly (Part 2.3). We have requested for contributions from experts for this chapter, and we have left the debate open for controversial opinions such as the classifi cation of facial SV (The Editors).

S.-K. Hann (�)Korea Institute of Vitiligo Research, Drs. Woo & Hann’s skin clinic, Yongsan-Gu, Seoul, South Koreae-mail: [email protected]

Contents

1.3.2.1 Epidemiology and Clinical Features ................. 42

Epidemiology and General Features ..................... 42Clinical Features ................................................... 42Precipitating Factors ............................................. 43

References ........................................................................... 43

1.3.2.2 Classifi cation, Course and Prognosis ................ 44

Classifi cation of Segmental Vitiligo on the Face (Hann) ................................................ 44Classifi cation of Segmental Vitiligo of the Face and Neck (Gauthier) ........................... 45Diagnosis, Course, and Special Locations ............ 45Treatment Overview .............................................. 47

References ........................................................................... 48

Core Messages

Segmental vitiligo (SV) shares some charac- ›teristics and can be associated with nonseg-mental vitiligo (NSV).However, SV is currently considered as a dis- ›tinct entity.SV usually has an early onset and it spreads ›rapidly in the affected dermatomal area.The hair follicle melanocytic compartment is ›frequently involved.The purpose of the classifi cation of SV, espe- ›cially facial, is to establish a prognosis.Multiple SV should be differentiated from NSV. ›Early medical and UV treatment of SV is rec- ›ommended based on recent data.

42 S.-K. Hann et al.

1.3.2.1Epidemiology and Clinical Features

Yvon Gauthier, Laila Benzekri, and Seung-Kyung Hann

It has been proposed by Koga in 1977 that two types of vitiligo exist from the pathophysiological and clinical points of view, each having a separate patho-genesis [10]. Nonsegmental vitiligo (NSV, Type A) associated with autoimmune disease and segmental vitiligo (SV, Type B) resulting from the dysfunction of sympathetic nerves in the affected area. In the two types, acquired melanocyte loss is a common feature. Based on pure clinical considerations, localized forms of vitiligo can be further subclassifi ed into focal, meaning one or more macules in one area, but not clearly in a segmental distribution (Chap. 1.2.1); segmental, one or more macules, more or less dis-tributed according to a zosteriform distribution; and mucosal, with isolated mucous membrane involve-ment (Chap. 1.3.4).

Epidemiology and General Features

The global incidence of vitiligo is generally acknowl-edged to be 0.5–1%. In published series of vitiligo, the percentage of SV varies from 5 to 7.9% [1, 6, 13] and up to 15% in our personal recruitment (Yvon Gauthier).

Korean studies have shown a range between 5.5 and 16.1% [14, 15].

Koga and Tango reported that SV affects, more commonly, the young and indicated a higher (27.9%) incidence in children [9]. In Hann and Lee’s report [6], SV developed before 30 years of age in 87.0% of cases, 41.3% were younger than 10 years, and the mean age of onset was 15.6 years. The earliest reported onset was immediately after birth, whereas the latest was 54 years. Most cases were less than 3 years in duration at referral, ranging from 2 months to 15 years. Delayed referral may refl ect the prevailing belief among doc-tors that no treatment is available for vitiligo.

Clinical Features

The typical lesion is not very different from the macule observed in NSC. The most common form is a totally amelanotic macule surrounded by normal skin. The color of the macule is usually pure white or chalk white. However, as in NSV, a multichrome variation of hypop-igmentation can be observed, and overall a less uniform depigmentation pattern was seen in SV compared to NSV when a decision was needed for grading a patch [16]. In some cases, such as fair skin, the lesions are not easy to see under normal light, but can be distinguish-able with Wood’s light examination. Similar to herpes zoster, the depigmented patches are confi ned to a defi -nite dermatome [3] (partial or complete involvement), but they also commonly overlap several dermatomes and sometimes may cross the midline (Fig. 1.3.2.1).

a b

Fig. 1.3.2.1. Typical aspect of SV of the face with poliosis crossing either slightly the mid line (a), or more markedly (b). (It cor-responds to Hann’s type I and Gauthier’s type IVC, see Figs. 1.3.2.3 and 1.3.2.4, respectively)

1.3.2 Segmental Vitiligo 43

In majority of cases, depigmentation spreads within the segment in a short period of time and then stops. It leaves the skin segment partially or totally depigmented. It is rare for a patient with SV to progress to the gener-alized form. Such a phenomenon is probably related to the rare association of SV and NSV called “mixed viti-ligo” [5]. In this case, the increased severity of SV ver-sus NSV in response to therapy suggests that the dosing of the predisposing skin anomaly is augmented in the SV area (Part 2.3).

The head is involved in more than 50% of cases. In decreasing order of frequency, the trunk, the limbs (Fig. 1.3.2.2), the extremities, and the neck are com-mon sites of involvement [1, 2, 4, 6, 7, 11]. In females, the neck is more frequently involved than the extremi-ties. Lerner [12] reported that SV occurs as a single lesion in 75% of patients, a fi nding confi rmed by the study of Hann and Lee [6] who found that 87% of patients had a single lesion.

There is no preferential distribution between right and left sides of the body. Some patients have lesions in two different unilateral dermatomes. The most com-monly involved is that of the trigeminal nerve [6–8].

Depigmentation of hair (poliosis, leukotrichia) occurs in vitiliginous macules. Poliosis has been shown to occur in 48.6% of cases of SV [6, 7]. Involvement is variable; a few to many hair of a single macule may be depigmented. The eyebrow and eyelashes are com-monly involved in SV located on the ophthalmic branch of the trigeminal nerve. Other involved hairy sites include scalp, pubis, and axillae.

A family history of vitiligo is present in approxi-mately 12% of SV cases [2, 6, 7]. El Mofty [4] and Koga [9] suggested that SV is not signifi cantly associ-ated with other autoimmune disease. This is still debated

because Park et al. [14] showed that about 9.5% of SV cases were associated with other diseases. Similarly, Hann and Lee [6] reported 6.7% of patients with associ-ated disease, either allergic or autoimmune. Common diseases associated with SV include atopic dermatitis, common in general in this age group, and halo nevus, which is associated to SV nearly as frequently as to NSV (discussed in Chap. 1.3.5 and Part 1.7). Considering the prevalence of allergic and autoimmune disorders among the general population, whether these fi ndings are pathogenically associated or coincidental is not established.

Precipitating Factors

The appearance of linear or macular depigmentation after scratches or traumas was not found by Koga [9] in SV outside the involved segment. However, other authors reported that sunburns, trauma, or local repeated pressures were locally aggravating factors in 4.8% of patients with SV [2].

References

1. Bang JS, Lee JW, Kim TH et al (2000) Comparative clinical study of segmental and non segmental vitiligo. Kor J Dermatol 38:1037–1044

2. Barona SK, Arruneteguy A, Falabella R (1995) An epide-miologic case-control study in a population with vitiligo. J Am Acad Dermatol 33:621–625

3. Bolognia JL, Orlow SJ, Glick SA (1994) Lines of Blasehko. J Am Acad Dermatol 31:157–190

4. El Mofty AM, El Mofty M (1980) Vitiligo: a symptom com-plex. Int J Dermatol 19:237–244

5. Gauthier Y, Cario Andre M, Taïeb A (2003) A critical appraisal of vitiligo etiologic theories. Is melanocyte loss a melanocytorrhagy? Pigment Cell Res 16:322–332

6. Hann SK, Lee HJ (1996) Segmental vitiligo: clinical fi nd-ings in 208 patients. J Am Acad Dermatol 35:671–674

7. Hann SK, Park SK, Chan WH (1997) Clinical features of vitiligo. Clinic Dermatol 15:891–897

8. Hann SK, Chang HJ, Lee HS (2000) The classifi cation of segmental vitiligo on the face. Yonsei Med J 41:209–212

9. Koga M, Tango T (1988) Clinical features and course of type A and type B vitiligo. Br J Dermatol 118:223–228

10. Koga M (1977) Vitiligo: a new classifi cation and therapy. Br J Dermatol 97:255–261

11. Lee SJ, Cho SB, Hann SK (2007) Classifi cation of vitiligo. In: Gupta S, Olsson M, Kanwar AJ, Ortonne JP (eds) Surgical management of vitiligo. Blackwell, Oxford, pp 20–30

12. Lerner AB (1959) Vitiligo. J Invest Dermatol 32:285–310

Fig. 1.3.2.2 SV of the left shoulder and upper limb


13. Ortonne JP (1983) Vitiligo and other hypomelanosis of hair and skin. Plenum, New York, pp 147–148

14. Park K, Youn JL, Lee YS (1988) A clinical study of 326 cases of vitiligo. Korean J Dermatol 26:200–205

15. Song MS, Hann SK, Ahn PS et al (1994) Clinical study of vitiligo: comparative study of type A and type B vitiligo. Ann Dermatol (Seoul) 6:22–30

16. Taïeb A, Picardo M; VETF Members (2007) The defi nition and assessment of vitiligo: a consensus report of the Vitiligo European Task Force. Pigment Cell Res 20:27–35

1.3.2.2Classifi cation, Course and Prognosis

Seung-Kyung Hann and Yvon Gauthier

Following Koga’s initial report [6] suggesting that SV and NSV were distinct vitiligo subtypes, further work confi rmed that a key aspect of these cases was that the lesions did not cross the midline and were distributed along a unilateral dermatome, thus enabling a prediction of the prognosis [2, 4, 7, 9]. SV does not always show classical dermatomal distribu-tion but affects usually only one segment of the integ-ument. The segment might be composed of several or parts of several adjacent dermatomes or have no rela-tionship to dermatomes at all, nor to any other lines such as Blaschko’s lines or acupuncture lines (see discussion in Part 2.3). The progression of SV, which is usually limited to months or a few years [2, 7], dif-fers remarkably from the chronic progressive course of NSV [1, 4, 9]. Since the face is a commonly involved site of vitiligo and the area that causes the most psychological impact, most patients are willing to undergo intensive treatment.

Therefore, knowledge about the exact spreading pattern and prognosis is important for both patients and doctors. The two following classifi cation schemes can help making predictions.

Classifi cation of Segmental Vitiligo on the Face (Hann)

The distribution of SV on the face is classifi ed into fi ve patterns (Fig. 1.3.2.3) [5]. Type Ia represents the lesion which initiates from the right side of the forehead, crosses the midline of the face and spreads down to the eyeball, nose and cheek of the left side of the face. Type Ib appears as a mirror image of Ia. The lesion starts from the left side of the face and spreads down the right side of the face, crossing the midline. In type II, the lesion starts from the area between the nose and lip, then arches to the preauricular area. In type III, the lesion initiates from the lower lip and spreads down to the chin and neck. In type IV, the lesion originates from the right side of the forehead and spreads down to


the eyeball, nose and cheek areas without crossing the midline. In type V, the lesion is confi ned to the left cheek area.

Some SV on the face cannot be classifi ed by this system. Type I is the most common and type V is the least common. There are no signifi cant differences in age, sex, duration of initial lesions, progression pat-tern, and clinical type among these classifi cations.

Classifi cation of Segmental Vitiligo of the Face and Neck (Gauthier)

Recently, we have proposed a new simplifi ed classifi -cation of SV of the face [10], which varies from the previous classifi cation by Hann et al. [5]. With this new system all SV of the face can be classifi ed. The sites involved by herpes zoster and SV were compared before establishing this new classifi cation. In 26% of cases, SV was distributed exactly to a trigeminal der-matome: ophthalmic (V1) maxillary (V2) mandibular (V3). In 64% of cases SV did not follow exactly der-matomes and was overlapping one, two or three der-matomes, as in many cases of facial herpes zoster. A classifi cation of facial SV according to fi ve topographic

patterns was thus proposed (Fig. 1.3.2.4): type I, corresponding to the V1 ophthalmic branch (partial or total involvement); type II, corresponding to the V2 maxillar branch (partial or total involvement); type III, corresponding to V3 mandibular branch (partial or total involvement); type IV, corresponding to mixed distribution on several dermatomes (4a = V1 + V2, 4b = V2 + V3, 4c = V1 + V2 + V3); and type V, cor-responding to the cervicofacial one.

Diagnosis, Course, and Special Locations

Most often SV patches remain unchanged for the rest of the patient’s life after rapid initial spreading in the affected segment [2]. However, rarely it can progress again after being quiescent for several years (Fig. 1.3.2.5). When SV progresses, it usually spreads over the predicted segment. However, in very rare cases, lesions may become generalized, a situation referred to as mixed vitiligo (Chap. 1.2.1). Early SV most often appears as a solitary oval shaped white macule or as a patch that is diffi cult to differentiate from “focal” vitiligo until proven by a subsequent typi-cal distribution pattern. A white macule on the nipple

Fig. 1.3.2.3 Hann’s classifi cation of segmental vitiligo of the face


Fig. 1.3.2.4 Gauthier’s classifi cation of segmental vitiligo of the face: I corresponding to V1; II corresponding to V2; III cor-responding to V3; IVa corresponding to mixed distributionV1 +

V2; IVb corresponding to mixed distribution V2 + V3; IVc cor-responding to mixed distribution V1 + V2 + V3; V correspond-ing to cervicofacial

Fig. 1.3.2.5 Recurrence of vitiliginous lesions at the periphery of an autologously grafted SV site. The graft was done 10 years before the recurrence (corresponds to Hann’s type II and Gauthier’s type III, see Figs. 1.3.2.3 and 1.3.2.4, respectively)

or areola appearing as the initial lesion can be assumed to be an early manifestation of SV (Fig. 1.3.2.6) [4]. Nipple or areolar involvement as the initial lesion in NSV is very rare and becomes bilateral later (Fig. 1.3.2.7). The repigmentation of vitiligo on nipple or areolar skin is diffi cult with UV or medical therapies. Instead, surgical treatment such as epidermal grafting can lead to complete repigmentation for both SV and NSV. If SV occurs bilaterally, following the same con-tralateral (or different) dermatomes, it may cause dif-fi culties in defi ning vitiligo type (Fig. 1.3.2.8). It may at some point be confused with NSV or, for some loca-tions, such as white patches of both legs, to piebaldism (Chaps. 1.2.1 and 1.3.11). Lee and Hann [8] reported that 5 out of 240 patients, who had SV, exhibited two different depigmented segments on the same or


Fig. 1.3.2.6 A white macule on the areola or nipple often progresses to segmental vitiligo (left panel early stage, diffi cult to predict; right panel, clear segmental involvement)

Fig. 1.3.2.7 Bilateral involvement of the nipple in NSV

opposite sites of the body. The clinical course of bilat-eral SV seems to be the same as unilateral SV although only fi ve cases have been followed for up to maximum of 3 years. In our experience, PUVA therapy and topi-cal steroid treatment can induce repigmentation or stop progression in bilateral SV.

Treatment Overview

SV was previously known to be resistant to treatment. However, recent studies reported surprisingly good results. SV at an early stage has an excellent progno-sis. Since the most frequently involved site of SV is the face, it can be easily detected and treated. Effective treatment modalities at an early stage are not stan-dardized but include topical steroids, topical calcineu-rin inhibitors or UV therapy, isolated or in combination (Fig. 1.3.2.9). Because SV often causes leukotrichia, it may resist standard medical therapies. Stable SV with leukotrichia can be cured successfully with epi-dermal grafting and subsequent UV treatment [3]. There may be also a possibility of activation and migration of epidermal melanocytes to the hair folli-cle. Overall, stable SV is a good indication for epider-mal grafting and can be cured almost completely without recurrence (for a discussion of surgical thera-pies, see Part 3.7).


Fig. 1.3.2.9 Almost complete repigmentation of SV after 4 months of 308 nm excimer laser treatment (a, before; b, after) (corre-sponds to Hann’s type I and Gauthier’s type IVa, see Figs. 1.3.2.3 and 1.3.2.4, respectively)

a b

a

b

Fig. 1.3.2.8 Bilateral segmental vitiligo of the same distribution in Asian (a) and black (b) patients

References

1. Bang JS, Lee JW, Kim TH et al (2000) Comparative clinical study of segmental vitiligo and non-segmental vitiligo. Korean J Dermatol 38:1037–1044

2. Hann SK, Lee HJ (1996) Segmental vitiligo: clinical fi nd-ings in 208 patients. J Am Acad Dermatol 35:671–674

3. Hann SK, Im S, Park YK, Hur W (1992) Repigmentation of leukotrichia by epidermal grafting and systemic psoralen plus UV-A. Arch Dermatol 128:998–999

4. Hann SK, Park YK, Chun WH (1997) Clinical features of vitiligo. Clinic Dermatol 15:891–897

5. Hann SK, Chang JH, Lee HS, Kim SM (2000) The classifi -cation of segmental vitiligo on the face. Yonsei Med J 41:209–212


6. Koga M (1977) Vitiligo: a new classifi cation and therapy. Br J Dermatol 97:255–261

7. Koga M, Tango T (1988) Clinical features and courses of type A and type B vitiligo. Br J Dermatol 118:223–228

8. Lee HS, Hann SK (1998) Bilateral segmental vitiligo. Ann Dermatol (Seoul) 10:129–131

9. Song MS, Hann SK, Ahn PS et al (1994) Clinical study of vitiligo: comparative study of type A and type B vitiligo. Ann Dermatol (Seoul) 6:22–30

10. Gauthier Y, Taieb A (2006) Proposal for a new classifi cation of segmental vitiligo of the face. Pigment Cell Res 19:515 (abstract)

Documents

Vitiligo || Segmental Vitiligo