Segmental vitiligo: Clinical findings in 208 patients

Segmental vitiligo: Clinical findings in 208 patients

CLINICAl. AND LABORATORY STUD--S III Segmental vitiligo: Clinical findings in 208 patients Seung Kyung Hann, MD, and H o Jung Lee, M D Seoul, Korea ...

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CLINICAl. AND LABORATORY STUD--S III

Segmental vitiligo: Clinical findings in 208 patients Seung Kyung Hann, MD, and H o Jung Lee, M D Seoul, Korea

Background: Vitiligo affects approximately 1% of the general population without racial, sexual, or regional differences. However, studies of segmental vitiligo have been few and the number of patients limited. Objective: The aim of this study was to analyze the clinical features of patients with segmental vitiligo. Methods: We evaluated sex, age at onset, age at initial visit, character of the initial lesion, status of progression, precipitating factors, involved sites, dermatomal distribution, family history, Koebner phenomenon, presence of pofiosis, dominant hand, and associated diseases. Results: Segmental vitiligo had an early onset, rapid progression, no specific precipitating factors, and linear spreading in the affected dermatomal area. The most commonly involved dermatome was the wigeminal. Only a few patients had an associated autoimmune disease. Conclusion: The clinical features of segmental vitiligo differ from those of nonsegmental vitiligo; pathogenesis may also differ. (J Am Acad Dermatol 1996;35:671-4.)

Vitiligo affects approximately 1% of the general population without racial, sexual, or regional differences. 1 The classification of vitiligo into two types, generalized and localized, is widely used. 2 Localized lesions in a dermatomal distribution that do not cross the midline can be further classified as segmental vitiligo. In 1977 Koga 3 proposed that the pathogenesis of the two types differ. The segmental type results from dysfunction of sympathetic nerves in the affected area, whereas the nonsegmental type is an immunologic disorder. The clinical features of generalized vitiligo have been frequently described, 1, 4-8 but few patients with segmental vitiligo have been studied. W e reviewed the clinical features of 208 patients with segmental vitiligo. MATERIAL AND METHODS

Of 1292 patients with vitiligo seen from 1992 to 1994, evaluations of 208 patients with segmental vifiligo were performed; data compiled were sex, age at onset, age at initial visit, character of the initial lesion, status of

From the Depatanaent of Dermatology, Yonsei University College of Medicine. Accepted for publication May 13, 1996. Reprint requests: Setmg Kytmg Harm, MD, Department of Dermatology, Yonsei University College of Medicine, C.P.O. Box 8044, Seoul, Korea. Copyright © 1996 by the American Academy of Dermatology, Inc. 0190-9622/96 $5.00 + 0 16/1/74896

progression at initial visit, precipitating factors, involved sites, dermatomal distribution, family history, Koebner phenomenon, presence of poliosis, dominant hand, and associated diseases. The character of the initial lesion was categorized as single or multiple. The dermatomal distribution was classifted according to Grant' s dermatomes 9 and dermatomes as modified by Haerer.10 When the lesion involved a certain dermatome along with other scattered lesions, the derrnatomal lesion was selected. When the lesion involved two distinct dermatomes, both were recorded. RESULTS

A g e at onset and age at initial visit O f the 208 patients with segmental vitiligo, 87 (41.8%) were male and 121 (58.2%) were female. The mean age at onset was 15.6 years (male patients, 13.9 years; female, 16.8 years). The age range of the largest group of patients was up to 9 years of age (Table I). The earliest onset was immediately after birth, whereas the latest was 50 years of age. Average age at first visit was 20 years (male patients, 17.3 years; female, 21.9 years). The youngest patient was 3 months old and the oldest was 55 years of age. Duration

The mean duration was 4.8 years (male patients, 4.2 years; female, 5.3 years); most cases (72.1%) were less than 5 years in duration, regardless of sex. The minimal and maximal durations were 1 month and 25 years, respectively. 671

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Table I. Age at onset Age (yr)

Male (%)

Female(%)

Total (%)

0-9 10-19 20-29 30-39 40-49 50-59 Total

39 (44.8) 28 (32.2) 13 (14.9) 6 (6.9). 1 (1.1) -87

47 (38.8) 31 (25.6) 23 (19.0) 12 (9.9) 7 (5.8) 1 (0.8) 121

86 (41.3) 59 (28.4) 36 (17.3) 18 (8.7) 8 (3.8) 1 (0.5) 208

Table II. Site of vitiligo Fig. 1. Segmental vitiligo distributed along thoracic and lumbar dermatomes (T10-12 and L1-2).

Character of initial lesion and status of progression at initial visit A total of 181 patients (87%) had a single initial lesion, whereas the other 27 (13%) had multiple lesions. At first visit, the lesion had progressed in 115 patients (55.3%), was stable in 85 (40.9%), and had regressed slightly without any treatment in 8 (3.8%). Site of involvement The face was the most common site of involvement, regardless of sex. The trunk, extremities, and neck, in descending order of frequency, were involved in men. In women, the neck was more frequently involved than the extrerm'ties (Table II). Dermatomal distribution Left side involvement (104 patients: male, 41; female, 63) was almost the same as fight side involvement (102 patients" male, 47; female, 55). Both sides were involved in two patients, and seven patients had lesions in two different unilateral dermatomes (Fig. 1). The trigeminal dermatome (Fig. 2) was most commonly involved in either sex. Other involved dermatomes include the thoracic, cervical, lumbar, and sacral dermatome in either sex, in descending order of frequency (Table l/I). Of the 133 patients who mentioned their dominant hand, eight were left-handed and 125 were fight-handed. The right side was involved in 57 of 125 right-handed patients, whereas the left side was involved in the remaining 68. Of the eight left-handed patients, four had left-side involvement and four, right-side involvement.

Site

Male (%) Female(%)

Head and neck Face Neck Scalp Trunk Chest and abdomen Back Extremities Upper limbs Lower limbs Total

57 (62.6) 49 (53.8) 7(7.7) 1 (1.1) 21 (23.1) 17 (18.7) 4 (4.4) 13 (14.3) 7 (7.7) 6 (6.6) 91

Total(%)

87 (65.9) 65 (49.2) 20(15.2) 2 (1.5) 34 (25.8) 31 (23.5) 3 (2.3) 11 (8.3) 7 (5.3) 4 (3.0) 132

144 (64.6) 114 (51.1) 27 (12.1) 3 (1.4) 55 (24.7) 48 (21.5) 7 (3.1) 24 (10.8) 14 (6.3) 10 (4.5) 223

Table III. Dermatomal distribution of vitiliginous lesions Dermatome

Male (%)

Female(%)

Total (%)

Trigeminal Cervical Thoracic Lumbar Sacral Total

49 (53.9) 12 (13.2) 19 (20.9) 10 (11.0) 1 (1.1) 91

65 (50.8) 26 (20.3) 31 (24.2) 4 (3.1) 2 (1.6) 128

114 (52.1) 38 (17.4) 50 (22.8) 14 (6.4) 3 (1.4) 219

Poliosis Poliosis was present in 101 patients (48.6%) (male, 38; female, 63). The eyebrows were the most commonly involved (45 patients [21.6%]). In men the involved sites were scalp hair (18.4%), eyebrows (17.2%), and pubic hair (2.3%), whereas in women the eyebrows (24.0%), scalp hair (14.9%) and pubic hair (2.0%) were involved. Both scalp hair and eyebrows were involved in 19 patients (9.1%). Associated diseases Fourteen patients (6.7%) had an associated disease suspected of being allergic or immtmologic. The most common was atopic dermatitis, which was

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Table IV. Associated diseases Disease

Atopic dermatitis Halo nevus Thyroid disease* Diabetes mellitus Alopecia areata Uveitis Total

No. of patients

7 2 2 1 1 1 14

*Thyroid diseases with proven or suspected immunologic cause

present in seven patients (3.4%). Other associated diseases are listed in Table IV. Other clinical characteristics

Twenty-four patients (11.5 %) had a family history of segmental vitiligo, and Koebner phenomenon was observed in 11 patients (5.3%). No particular precipitating factors were identified.

Fig. 2. Segmental vitiligo distributed in ophthalmic and maxillary branches of Irigeminal dermatome.

DISCUSSION

The clinical features of segmental vitiligo differ from those of nonsegmental vitifigo. 11 Segmental vitiligo usually has an early onset and spreads rapidly in the affected dermatomal area. The depigmented patches then persist unchanged for the rest of the patient's life. Therefore patients with stable segmental vitiligo are good candidates for treatment with epidermal grafting. 12, 13 Of patients with vitiligo the percentage with the segmental type varies. E1 Mofty and E1 Mofty 14 reported 5%; Koga and Tango 11 27.9%; in previous Korean studies, 5.5% to 9.5% 5, 8; and in our study 16.1%. Vitiligo may develop at any age, but usually begins at 10 to 30 y e a r s o f age. 1'4'15 However, according to an epidemiologic study by Howitz et al., 16 approximately 50% of the patients in whom vitiligo developed were older than 40 years of age. Koga and Tango 11 reported that the onset of nonsegmental vitiligo could occur at any age, whereas segmental vitiligo generally affected the young. In our study, segmental vitiligo began before 30 years of age in 87.0% of our patients, and before the age of 10 years in 41.3 %. The mean age at onset was 15.6 years in our study, which is younger than the 23.1 years reported by Levai17; 24.2 years by Seghal4; 23.8 years by Park, Youn, and Liras; 24.1 years by Harm et al.6; and 19.6 years by Park, Youn, and Lee. 8 The most commonly involved sites of nonseg-

mental vitiligo are exposed areas such as the face and dorsum of the hands. In our study of segmental vitiligo, the involved sites were the face, trunk, neck, extremities, and scalp, in descending order of frequency. Lerner is reported that 75% of cases of segmental vitiligo are single lesions. We found these in 87% of our patients. In the 31 patients with segmental vitiligo studied by Park, Youn, and Lee, 8 the abdomen, neck, face, and chest, in descending order of frequency were most commonly involved. This report differs from our results. Poliosis has been associated with vifiligo in 8.9% to 45% of patients. 4' 6, 17 It occurred in 48.6% of our patients. The eyebrows and the scalp hair were most commonly involved (46.7%). Trauma, sunburn, psychologic stress, pregnancy, and contraceptives may be the precipitating factors of nonsegmental vitiligo. However, unlike other reports, 4, 7, 11 we found sunburn, trauma, or pregnancy as aggravating factors in only 10 patients. Family history was present in 11.5%, shmilar to the 7.4% reported by Park, Youn, and Lim 5 and the 12% reported by Hann et al.6 E1 Mofty and E1 Mofty 14 claimed that segmental vitiligo is not associated with other autoimmune diseases, but Park, Youn, and Lee s found that it was in approximately 9.5% of their patients. Koga and Tango 11 found that disease with an autoimmune pathogenesis occurred more frequently in patients with nonsegmental vitiligo than in those with seg-

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mental vitiligo. In our study, association with thyroid disease, diabetes melfitus, pernicious anemia, or halo nevus was found in only 3.4% of patients; this percentage was lower than those in other reports.4' 7, 11 However, we cannot conclude that an autoimmune mechanism is restricted to nonsegmental vitiligo because systemic and topical steroid treatment and PUVA therapy can inhibit spreading and induce repigmentation of new lesions of segmental vitiligo, especially on the face (unpublished data). REFERENCES

1. Lemer AB. Vitiligo. J Invest Dermatol 1959;32:285-310. 2. Ortonne JP, Moscher DB, Fitzpatrick TB. Hypomelanotic disorders in vitiligo and other hypomelanoses of hair and skin. New York: Plenum, 1983:129-310. 3. Koga M. Vitiligo: a new classification and therapy. Br J Dermatol 1977;97:255-61. 4. Seghal VN. A clinical evaluation of 202 cases of vitiligo. Cuffs 1974;14:439-45. 5. Park SY, Youn JI, Lim SD. A clinical study of 217 cases of vifiligo. Korean J Dermatol 1981; 19:145-52. 6. Hann SK, Park YK, Whang KC, et al. Clinical study of 174 patients with generalized vitiligo. Korean J Dermato11986; 24:798-805.

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7. Halm SK, Song MS, Park YK, et al. Childhood vifiligo: clinical study compared with adult vifiligo. Ann Dermatol 1991 ;3:112-8. 8. Park KC, Youn JI, Lee YS. Clinical study of 326 cases of vifiligo. Korean J Dermatol 1988;26:200-5. 9. Grant JCB, editor. Grant's Atlas of anatomy. Baltimore: Williams & Wilkins, 1972:663-5. 10. Haerer AF, editor. Dejong's The neurologic examination. Philadelphia: JB Lippincott, 1992:55-64. 11. Koga M, Tango T. Clinical features and course of type A and type B vitifigo. Br J Dermatol 1988;118:223-8. 12. Harm SK, Im S, Park YK, et al. Repigrnentation of leukotrichia by epidermal grafting and systemic psoralen plus UV-A. Arch Dermatol 1992;128:998-9. 13. Hann SK, Im S, Bong HW, et al. Treatment of stable vitiligo with autologous epidermal grafting and PUVA. J Am Acad Dermatol 1995;32:943-8. 14. E1Mofty AM, E1Mofty M. Vitiligo: a symptom complex. Int J Dermatol 1980;19:238-47. 15. BeN PN, Bhatia RK. 400 cases of vifiligo: a clinicotherapeutical analysis. Indian J Dermatol 1972;17:51-6. 16. Howitz J, Brodthagen H, Schwartz M, et al. Prevalence of vifiligo:epidemiologic survey on the Isle of Borholrn, Denmark. Arch Dermatol 1977; 113:47-52. 17. Levai M. A study of certain contributory factors in the development of vitiligo in South Indian patients. Arch Dermatol 1958;78:364-70. 18. Lemer AB. On the etiology of vitiligo and gray hair. Am J Med 1971;51:147-56.