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U.Yu. Sabirov

Republican specialized scientific-practical medical center of Dermatovenereology and Cosmetology

A.S. Yakubova

Republican Specialized Scientific and Practical Medical Center for Dermatovenereology and Cosmetology of the Ministry of Health

S.S. Arifov

Center for the Development of Professional Qualifications of Medical Workers of the Ministry of Health

Clinical features of segmental and non-segmental vitiligo

Authors:

U.Yu. Sabirov, A.S. Yakubova, S.S. Arifov

More about the authors

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To cite this article:

Sabirov UYu, Yakubova AS, Arifov SS. Clinical features of segmental and non-segmental vitiligo. Russian Journal of Clinical Dermatology and Venereology. 2021;20(6):140‑144. (In Russ., In Engl.)
https://doi.org/10.17116/klinderma202120061140

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Vitiligo is an acquired disease characterized by the occurrence of well-defined white or milky-white depigmented patches on the skin, varying in size and shape and tending to increase in size over time. Globally, the prevalence of vitiligo in the population is about 1%; it occurs in different age groups in people with all skin types [1, 2].

The increasing incidence of vitiligo among children, young people, and working-age individuals makes the disease of significant social importance. Traditional misconceptions about the nature of this disease lead to negative public attitudes toward people with white spots on the skin, and the persistent and long-lasting course of the disease and the low effectiveness of treatment increases its social significance [3].

The etiology and pathogenesis of vitiligo are not fully understood. The depigmentation is due to the destruction or complete disappearance of pigment cells, melanocytes, in the skin.

Currently, the most commonly accepted theories of vitiligo origin are the neurogenic, endocrine, immune, and melanocytes self-destruction theories. In addition, some other significant factors have been identified contributing to vitiligo occurrence: ultraviolet radiation, chemicals, viral infections, oxidative stress, antioxidant defense state, etc. Some authors adhere to the theory of the multifactorial nature of vitiligo [4—8].

The presentation and course of the disease are well described in the literature. There are several clinical classifications of vitiligo. For instance, A.B. Lerner (1959) suggested considering 3 types of vitiligo [5]:

(a) Segmental, localized or focal vitiligo, localized along the nerves and nerve plexuses;

b) Generalized or vulgar vitiligo localized to the face, arms, armpits, trunk, limbs and other parts of the body;

c) Total or universal vitiligo affecting all or most of the skin.

J.P. Ortonne et al. proposed the following vitiligo classification [9]:

1) Localized type with the following varieties: focal vitiligo (one or more patches in the same area); segmental vitiligo (one or more patches are located along the nerves or nerve plexuses); mucosal vitiligo (only the mucous membranes are affected);

2) Generalized type with the following varieties: acrofacial (affecting the face and distal parts of the hands and feet); vulgar (many randomly-localized patches); mixed (combination of vulgar and acrofacial or vulgar, segmental and acrofacial varieties);

3) Universal type (total or almost total depigmentation of the whole skin).

For several decades, the clinical classification has been used developed by D.B. Moscher, in which three types of vitiligo are distinguished [10]:

1) localized type including focal vitiligo (one or more patches in the same area); segmental vitiligo (patches along the nerve or nerve plexus) and mucosal vitiligo (only mucosal membranes are affected);

2) generalized type including acral or acrofacial vitiligo (face, hands, feet are affected); vulgar (multiple randomly-localized patches) and universal (total or almost total depigmentation of the skin);

3) Sutton disease or Sutton nevus (skin depigmentation around the nevus) [10].

M. Koga and T. Tango suggested distinguishing between two types of vitiligo. In type B (segmental), depigmented patches are located along the nerves or nerve plexuses. Type A (nonsegmental) includes all other types of vitiligo: focal, generalized (vitiligo vulgaris), acrofacial, and universal type, as well as mucosal vitiligo [11]. According to some authors, segmental and nonsegmental types of vitiligo have different pathogenesis and genetic basis of the disease [9, 10, 12—16].

The objective is to study the differences in the clinical presentation and course of segmental and nonsegmental types of vitiligo.

We followed up 91 patients with vitiligo aged 9 to 65 years (mean age 42±1.9 years), including 47 (51.6%) females and 44 (48.4%) males (Table 1). In females, vitiligo was more common between the ages of 11 and 20 years, whereas in males it was more common between the ages of 21 and 30 years.

Table 1. Patients’ split by gender and age

Age, years

Females

Males

Total

abs.

%

abs.

%

abs.

%

Up to 10

1

1.1

3

3.3

4

4.4

11–20

21

23.1

8

8.8

29

31.9

21–30

10

1.1

16

17.6

26

28.6

31–40

3

3.3

5

5.5

8

8.8

41–50

5

5.5

5

5.5

10

11

51–60

5

5.5

4

4.4

9

9.9

61 and over

2

2.2

3

3.3

5

5.5

Total

47

51.6

44

48.4

91

100

In 16 (17.6%) patients, the duration of disease was less than one year, in 54 (59.3%) patients 1–5 years, in 14 (15.4%) 6–10 years, in 7 (7.7%) over 11 years. Working-age individuals comprised 54 (59.3%) patients.

The distribution of patients according to the disease type is presented in the Table 2.

Table 2. Patients’ split by vitiligo type (according to M. Koga and T. Tango classification)

Age, years

Females

Males

Total

abs.

%

abs.

%

abs.

%

Segmental vitiligo

16

17.5

10

11

26

28.6

Nonsegmental vitiligo, including subtypes:

32

34.1

33

37.3

65

71.4

generalized

18

19.8

17

18.7

35

38.5

acrofacial

7

7.7

10

11

17

18.7

universal

3

3.3

1

1.1

4

4.4

focal

4

4.4

5

5.5

9

9.9

Total

48

51.6

43

48.4

91

100

As Table 2 shows, 65 (71.4%) patients had nonsegmental vitiligo, and 26 (28.6%) had a segmental type. The ratio of nonsegmental to segmental type was 2.5:1.

According to our observations, in 20 (77%) patients with the segmental type of vitiligo, the disease manifested before the age of 30 years. Thus, depigmented patches occurred before 10 years of age in 5 patients, before 20 years of age in 6 patients, and before 3 years of age in 9 patients.

The medical history of the patients was analyzed: 69 (75.8%) patients indicated factors that they thought might have triggered the onset of the disease; the remaining 22 (24.2%) patients could not name the cause of the disease.

Trauma (21.5% of patients), familial predisposition to vitiligo (16.9% of patients), and the presence of internal organ diseases (6.2%) were the most common triggers of nonsegmental vitiligo (Table 3). Patients with the nonsegmental vitiligo type more often associated the onset of disease with neuropsychological and emotional overstrain (69.2% of cases).

Table 3. The vitiligo provoking factors according to anamnestic data of patients with segmental and non-segmental vitiligo

Triggering factors for vitiligo

Segmental type (n=26)

Nonsegmental type (n=65)

abs.

%

abs.

%

Neuropsychic and emotional overstrain

18

69.2

11

16.9

Trauma

3

11.5

14

21.5

Familial predisposition to vitiligo

1

3.8

11

16.9

Internal organs diseases

1

3.8

4

6.2

Helminthosis

0

3

4.6

Intoxication

0

3

4.6

Total

23

88.5

46

50.5

As Table 3 shows, the nonsegmental vitiligo type occurred after trauma in more than 20% of patients, i.e., due to the Koebner phenomenon. It's been suggested that traumatic injury can lead to the secretion of neuropeptides in the skin that trigger melanocyte death. In addition, family cases of vitiligo in 16.9% of patients with nonsegmental type suggest the genetic etiology of the disease [14, 16]. Whereas in the segmental vitiligo, the frequent association of disease onset with neuropsychological and emotional overstrain is explained by the neurogenic genesis and dysfunction of the sympathetic nervous system.

Some comorbidities were identified during a thorough clinical, laboratory, and instrumental examination of the patients and their visits to physicians of related specialties. The frequency of the comorbidities depended on the type of vitiligo (Table 4).

Table 4. Concomitant diseases identified in patients with segmental and non-segmental vitiligo

Comorbidities

Segmental type (n=26)

Nonsegmental type (n=65)

abs.

%

abs.

%

Neurological disorders

7

26.9

6

9.2

neurasthenia

3

2

vegetovascular disorders

4

4

Gastrointestinal diseases

3

11.5

11

16.9

chronic cholecystitis

1

3

cholecystitis

1

2

gastritis

1

4

stomach ulcer

1

Endocrine disorders

3

11.5

8

12.3

hypothyroidism

3

4

diabetes mellitus

2

autoimmune thyroiditis

2

Gynecological diseases

2

7.7

3

4.6

ovarian cyst

1

1

adenomyosis

1

1

female infertility

1

Anemia

1

3.8

2

3.1

Skin conditions

2

3.1

alopecia

1

discoid lupus erythematosus

1

Total

16

61.5

32

49.2

Note. Twenty-six patients with segmental type and 65 patients with nonsegmental type of vitiligo were taken as 100%.

Table 5. Localization of lesions and the incidence of leukotrichia in patients with vitiligo

Patches location

Segmental type (n=26)

Nonsegmental type (n=65)

number of patches

%

number of patches

%

Scalp

2

7.7

4

6.1

Face

9

34.6

2

3.1

Neck

1

3.8

1

1.5

Trunk

5

19.2

24

36.9

Upper limbs

4

15.4

13

20.0

Lower limbs

6

23.1

15

23.1

Leukotrichia

14

53.8

7

10.8

Note. Twenty-six patients with segmental type and 65 patients with nonsegmental type of vitiligo were taken as 100%.

Neurological diseases were more common in the segmental type of vitiligo than in the nonsegmental type, in 26.9% and 9.2% of patients, respectively. Gastrointestinal tract diseases were identified more frequently in nonsegmental vitiligo patients (16.9%) versus segmental vitiligo patients (11.5%). It is known that nonsegmental vitiligo type is often associated with autoimmune diseases, such as diabetes mellitus, pernicious anemia (Addison-Biermer disease), autoimmune thyroiditis, etc. [17, 18]. Our studies are consistent with the data of other authors; autoimmune diseases (diabetes mellitus, autoimmune thyroiditis, alopecia, and discoid lupus erythematosus) were identified in 6 patients with nonsegmental vitiligo type, while in patients with segmental type, they did not occur.

The analysis showed that patches on the face (34.6%) and leukotrichia (53.8%) were more common in the segmental type of vitiligo, whereas in the nonsegmental type, patches were more often localized on the trunk (36.9%) and limbs (43.1%), and leukotrichia occurred less frequently (10.8%).

Thus, our clinical observations indicate that segmental and nonsegmental vitiligo differ significantly from each other according to many parameters (age of vitiligo onset, triggering factors, localization of patches, presence of leukotrichia foci, comorbidities), which should be considered during treatment and follow-up of patients.

The authors declare no conflict of interest.

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