Epidemiology of Childhood Vitiligo: A Study of625 Patients from North India

Sanjeev Handa, M.D., M.N.A.M.S., and Sunil Dogra, M.D., D.N.B.

Department of Dermatology, Venereology, and Leprology, Postgraduate Institute of Medical Education andResearch, Chandigarh, India

Abstract: To study the clinical and epidemiologic profile of childhoodvitiligo, we retrospectively analyzed the data of children with vitiligoattending the pigmentary clinic of our center. Of the 625 children seen over10 years, 357 (57.1%) were girls and 268 (42.9%) were boys. As compared toadult patients with vitiligo, this sex difference was found to be statisticallysignificant (p < 0.001). The mean age of onset of the disease was 6.2 years.Vitiligo vulgaris (generalized vitiligo) was the most common type, followedby focal, segmental, acrofacial, mucosal, and universal, in that order. Themost frequent site of onset was the head and neck, followed by the lowerlimbs, trunk, upper limbs, and mucosae. Leukotrichia was present in 77patients (12.3%), while Koebner phenomenon was observed in 71 patients(11.3%). Halo nevi were observed in 29 patients (4.4%). Seventy-six patients(12.2%) had a family history of vitiligo. Eight patients (1.3%) had an asso-ciated autoimmune disease. These associated disorders were alopeciaareata in two patients, and diabetes mellitus, thyroid disease, Addison dis-ease, polyglandular syndrome, and pemphigus vulgaris in one patient each.

Vitiligo is an acquired, pigmentary disorder charac-terizedbya loss ofmelanocytes resulting inwhite spots orleukoderma. It affects 0.1–4% of the population world-wide (1–5). One of the largest Indian studies reported aprevalence of 2.5% (6). Vitiligo usually begins in child-hood or young adulthood, with approximately half ofthe patients having onset prior to the age of 20 years.There are very little published data on the epidemiologyof childhood vitiligo worldwide (7,8) and only one studyon a small number of patients from India (9). Vitiligobeginning in childhood can be associatedwith significantpsychological trauma that may have long-lasting effectson the self-esteem of these children. Considering theextent of the disease burden and the scanty publishedinformation on the subject, we analyzed the records of

children with vitiligo in an effort to learn more about itsepidemiology and clinical features.

METHODS

The Postgraduate Institute of Medical Education andResearch (PGIMER), Chandigarh India, is a tertiarycare institute serving the population of northern India. Aretrospective analysis was made of the patient records atthe pigmentary clinic of the institute over the last 10 years(1990–1999). All patients £ 12 years of age with vitiligoreferred to the clinic were included in the study. Thediagnosis was made by experienced physicians and wasessentially clinical. Demographic details of all patientsincluding the age of onset, initial site of onset, durationof

Address correspondence to Sanjeev Handa, M.D., Departmentof Dermatology, Venereology, and Leprology, PGIMER, Chand-igarh – 160 012, India, or e-mail: handa_sanjeev@yahoo.com.

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disease, percentage of body area involved, associatedmucosal involvement, presence of leukotrichia, Koebnerphenomenon, halo nevi, associated diseases, and familyhistorywere obtained from the clinic notes. A recordwasmade of the precise distribution of lesions using the ruleof nine, and the cases were classified into six groupsaccording to the standard working classification of clin-ical types of vitiligo (10).

RESULTS

Of the 112,785 new patients who attended the skin out-patient department over a period of 10 years, 2672 hadvitiligo (relative incidence of 2.4%). Of the 2672 vitiligopatients, 625 (23.4%) were children (£ 12 years). Of the625 children, 210 (33.6%) were 0–6 years of age and 415(66.4%) were 6–12 years of age. Of the 625 children,357 (57.1%) were girls and 268 (42.9%) were boys. Incomparison to the adult patients with vitiligo this sexdifference was found to be statistically significant(p < 0.001). Duration of the disease at the time of pre-sentation varied from 1 month to 3 years. One hundredand two (16.3%) patients had onset between the age of0 to 4 years, 308 (49.3%) between 4 to 8 years and 215

(34.4%)between8 to 12years of age.The clinical types ofvitiligo observed in these children are depicted inTable 1.

Vitiligo vulgaris (generalized vitiligo) (Fig. 1) was themost common type, followed by focal, segmental (Fig.2), acrofacial (Fig. 3), mucosal, and universal, in thatorder.

The most common site of onset was the head andneck, followed by the lower limbs, trunk, upper limbs,and mucosae (Table 2). Six hundred and four patients

Figure 2. Segmental vitiligo on the face is a common site ofinvolvement.

Figure 1. Vitiligo vulgaris lesions on the face.

TABLE 1. Types of vitiligo in children

Clinical types Girls Boys Total (%)

Vulgaris 283 207 490 (78.4)Focal 54 36 90 (14.4)Segmental 13 16 29 (4.6)Acrofacial 6 4 10 (1.6)Mucosal 1 3 4 (0.6)Universal 0 2 2 (0.3)

Total 357 268 625 (100)

Figure 3. Acrofacial vitiligo lesions on the hands and lips.

208 Pediatric Dermatology Vol. 20 No. 3 May/June 2003

(96.4%) had less than 20% of the body area involved. Amajority of them (89.7%) had less than 5% body areainvolvement. Besides the mucosal form of vitiligo, asso-ciated involvement of mucosae with other clinical vari-ants (vulgaris or acrofacial) was seen in 37 patients(6.0%). The oral mucosa was involved in 37 patients(84.0%) and the genital/anal mucosa was involved in 7patients (15.9%). Leukotrichiawas present in 77 patients(12.3%),whileKoebnerphenomenonwasobserved in 71(11.3%) and halo nevi were seen in 29 (4.4%).

Seventy-six patients (12.2%) had a family history ofvitiligo. First-degree relatives (parents/siblings) wereaffected in 27 patients (35.5%) and second-degree rela-tives (grandparents, uncles, or aunts) in 49 patients(64.5%).Of these 76patients, 6hadmore thanone familymember with the disease. A family history of otherautoimmune diseases in first-degree relatives was presentin 25 patients (4%). Its frequency in second-degree rel-atives could not be analyzed due to incomplete infor-mation. Eight children (1.3%) had an associatedautoimmune disease. These associated disorders werealopecia areata in two patients, and diabetes mellitus,thyroid disease, Addison disease, polyglandular syn-drome, and pemphigus vulgaris in one patient each.

DISCUSSION

Worldwide clinicoepidemiologic data on childhoodvitiligo are scarce. Vitiligo is common in India, having aprevalence of 0.46–8.8% (6). But there is only a singlestudy on childhood vitiligo, from southern India,reporting aprevalence of 2.6%(9).Vitiligo affected 2.4%of the dermatology outpatients in this study.Of a total of2672vitiligopatients, 625 (23.3%)were children,which isin agreement with an earlier figure of 26% reported byJaisankar et al (9).More than 50% of the patients in ourstudy had onset of the disease between 4 and 8 years ofage (mean 6.4 ± 1.2 years). The peak onset has variedamong different studies, with many authors stating thatmost cases are acquired early in life, between 4 and 12years of age (8). An earlier study of 1436 vitiligo patientsfrom our institute reported that the disease starts at ayounger age in Indian patients (6). When the age atpresentation was studied, most were between 9 and 12

years of age. This difference between age of onset and ageof presentation could be because patients/parents mightignore the initial lesions and seek treatment only after thedisease progresses.

The prevalence of vitiligo was found to be higher ingirls than in boys (57.1% versus 42.9%) (p < 0.001).One explanation could be that parents worry more andtend to seek treatment for cosmetically disfiguring,depigmenting patchesmore frequently in girls. However,in earlier reported series (7,9,11), the majority of cases inthe pediatric age group were in girls.

In earlier published reports on childhood vitiligo (7,9),vitiligo vulgaris was the most frequent type reported. Inour study as well, this was themost common clinical type,seen in 490 patients (78.4%). As in Halder et al (7), focalvitiligowas the secondmost commonpresentation seenbyus. However, Jaisankar et al (9), in their study of 90 chil-dren, reported segmental vitiligo as the second most fre-quent presentation, occurring in 21% of patients, closelyfollowed by focal vitiligo in 20.1%. The percentage ofsegmental vitiligo has been reported to vary from 19% to21% in children (7,9). Segmental vitiligo was seen in only4.6% of our patients. Universal vitiligo was the leastcommon type seen in our study population. Unlike in ourstudy, Halder et al (7) and Jaisankar et al (9) reported theacrofacial form to be the least common. These differencescould be due to racial or even genetic factors, since theIndian subcontinent population is very diverse and skincolor varies widely, from fair- to dark-skinned individualsas we go from the north to the south of the country. Weobserved mucosal vitiligo in only 4 patients (0.6%),though 37 patients (6%) had associated mucosal in-volvement with other clinical types of vitiligo. Jaisankar etal (9) reportedaconsiderablyhigherfigureof 13.8%,whileHalder et al (7) had no patients with mucosal vitiligo.

The head and neck area was the most common site ofonset and themost commonsite at presentationobservedbyus. Jaisankaretal (9) reported thevarious sitesofonsetto be the lower limbs, head and neck, upper limbs, andthorax, in that order. Leukotrichia has been reported in3.7–4.4% of pediatric patients with vitiligo (7,9). It wasseen in 77 of our patients (12.3%),most commonly in thevitiligo vulgaris variant (84%), followed by focal andsegmental vitiligo. Koebner phenomenon has been re-ported to occur in as many as 33% of all vitiligo patients(6). This figure was only 11.3% in our pediatric patients.Its exact prevalence in the pediatric age group is notknown.We thoughtourfigureswere rather lowbecause achildwithvitiligo ismore likely tosubjecthisorher skin totraumawhile playing and hence is more likely to developkoebnerization of the skin. Halo nevi are reported tooccur in 0.5–14%of vitiligo patients in all age groups (6).We observed halo nevi in just 4.4% of our children.

TABLE 2. Sites of onset

Site Number (%)

Head and neck 315 (50.4)Lower limbs 122 (19.5)Trunk 109 (17.4)Upper limbs 71 (11.4)Mucosae 8 (1.3)

Total 625 (100)

Handa and Dogra: Childhood Vitiligo 209

Twelve percent of the children with vitiligo had afamily history of the disease. In a study from HowardUniversityHospital, familyhistoryof vitiligowaspresentin 35% of children (7). Similar to our results, anotherstudy from India (9) also reported a relatively lower fig-ure of 3.3%. The reported frequency of associatedautoimmune disorders in children with vitiligo is signi-ficantly less than that observed in the adult vitiligo pop-ulation. In our study 7 of 625 children (1.1%) hadassociated autoimmune diseases. Jaisankar et al (9)reported no other autoimmune disorders in theirpatients, while Halder et al (7) reported two cases ofalopecia areata in 82 childrenwith vitiligo.None had anyother autoimmune/endocrine disorder. Schallreuter et al(11) reported an increased frequencyof thyroid disease inchildren with vitiligo. Family history of other autoim-mune and/or endocrine diseases in first-degree relativeswas present in 25 children (4.0%) with vitiligo. It hasbeen demonstrated that there is a statistically significantincreased frequency of autoimmune diseases in the first-and second-degree relatives of children with vitiligo ascompared to adults with vitiligo. However, we could notanalyze the incidence of autoimmune diseases in second-degree relations due to incomplete information.

CONCLUSION

The body of clinicoepidemiologic data on children withvitiligo from our study is one of the largest reported thusfar.Vitiligo in the pediatric population (less than 14 yearsold) seems to be relatively common, contributing 26%ofall patients with vitiligo. The peak age of onset of the

disease was between 4 and 8 years, and a majority of thechildren had limited disease involving less than 5% oftheir body area. Based on our observations, we believethat vitiligo behaves essentially the same way in childrenas in adults, except that it is more predominant in girls,has lessmucosal involvement, and is less often associatedwith autoimmune/endocrine diseases.

REFERENCES

1. Lerner AB. Vitiligo. J Invest Dermatol 1959;32:285–310.2. Lerner AB, Nordlund JJ. Vitiligo. What is it? Is it

important? JAMA 1978;239:1183–1187.3. Howitz J, Brodthagen H, Schwartz M, Thomsen K.

Epidemiological surveyon the IsleofBornholm,Denmark.Arch Dermatol 1977;113:47–52.

4. Bolognia JL, Pawelek JM.Biology of hypopigmentation. JAm Acad Dermatol 1988;19:217–255.

5. Mosher DB, Fitzpatrick TB, Hori Y, Ortonne JP.Disorders of pigmentation. In: Fitzpatrick TB, Eisen AZ,Wolff K, Freedberg IM, Austen KF, eds. Dermatology ingeneral medicine. New York: McGraw Hill, 1993:903.

6. Handa S,Kaur I.Vitiligo: clinical findings in 1436 patients.J Am Acad Dermatol 1999;26:653–657.

7. Halder RM, Grimes PE, Cowan CA, Enterline JA,Chakrabarti SG, Kenney JA. Childhood vitiligo. J AmAcad Dermatol 1987;16:948–954.

8. Halder RM. Childhood vitiligo. Clin Dermatol 1997;15:899–906.

9. Jaisankar TJ, Baruah MC, Garg BR. Vitiligo in children.Int J Dermatol 1992;31:621–623.

10. Kovacs SO. Vitiligo. J Am Acad Dermatol 1998;38:647–666.

11. Schallreuter KU, Mann M, Weintraub R, Hashimoto K.Focal dermal hypoplasia presenting with an initial inflam-matory stage. Pediatr Dermatol 1991;7:278–282.

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