CLINICAL PROFILE OF SEGMENTAL VITILIGO IN A GROUP OF PAKISTANI PATIENTS.
Keywords: Clinical profile, Segmental vitiligo, Pakistan.
Vitiligo is an idiopathic, often heritable, acquired depigmenting disorder of skin characterized by, progressive, circumscribed hypomelanosis of the skin and hair, with total absence of melanocytes microscopically1-2. It affects 0.5-1% of the population worldwide without racial, regional or gender differences2-3. Vitiligo is classified into two main types i.e. generalized and localized. Localized vitiligo is further classified into focal and segmental vitiligo. Localized vitiligo with dermatomal distribution is called segmental vitiligo1-2,4. There is marked clinical difference between segmental vitiligo and non segmental vitiligo5. Clinical characteristics of vitiligo have frequently been described3,6-9 but only few studies have focused on segmental vitiligo10-12. Moreover important racial differences may exist in different communities.
Clinical characteristics of segmental vitiligo have never been described in Pakistani population. The purpose of this study was to describe the clinical profile of segmental vitiligo in a group of Pakistani patients.
PATIENTS AND METHODS
This decriptive study was conducted at Dermatology department of Combined Military Hospital Kohat, during a period of two years from September 2016 to August 2018. All the patients with clinical diagnosis of segmental vitiligo reporting to Dermatology outpatient during this two years period were included in the study after taking informed consent. Diagnosis of segmental vitiligo was based on the clinical findings of acquired, well-demarcated white patches distributed in a segmental/dermatomal pattern with no associated inflammation. Diagnosis was further confirmed by Wood's lamp examination. Clinical profile of segmental vitiligo which included the age of the patient, gender, age at onset, duration of the disease at presentation, affected dermatome, family history, presence or absence of Koebner phenomenon and the presence of associated diseases, was studied in these patients. Total number of patients with diagnosis of vitiligo regardless of type of vitiligo was also noted.
The study was approved by the Ethical and Scientific Committee of the Hospital. A total of 56 patients with segmental vitiligo were enrolled. Each patient was interviewed in detail and a thorough physical examination was performed with a focus on morphology and distribution of lesions. Data regarding the age, gender, age at onset, duration of disease at presentation, affected dermatome, family history, presence or absence of Koebner phenomenon and the presence of associated diseases was collected for each patient. Data for each patient was collected on a specially designed proforma for each patient separately. Descriptive statistics (mean with standard deviation, percentages and frequency distribution) were used to evaluate the results. Frequencies and percentages were calculated for categorical variables while mean and standard deviations were calculated for numerical variables. Computer program SPSS-14 was used to manage and analyze the data.
Table-I: Age at onset.
###Number of patients
Age at onset###Male###Female###Total
###(%age for gender)###(%age for gender)###(% age for total patients)
Onset during first decade###28(84.85%)###16(69.56%)###44(78.57%)
Onset during second decade###5(15.15%)###7(30.43%)###12(21.43%)
Onset during first 5 years of age###13(39.39%)###3(13.04%)###16(28.6%)
Onset between 5 to 10 years of age###15(45.46%)###13(56.52%)###28(50%)
Onset between 10 to 15 years of age###4(12.12%)###7(30.43%)###11(19.6%)
Onset between 15 to 20 years of age###1(3.03%)###Nil###1(1.8%)
Table-II: Frequency of body region and dermatome involved by segmental vitiligo.
Body region involved###Upper limbs###6###10.7
###Ophthalmic N division###8###14.3
A total of 56 patients with segmental vitiligo were enrolled. Out of these fifty six patients, 33 (58.9%) were male and 23 (41.1%) were female. The male to female ratio was 1.43:1. Age at presentation ranged from 2 years to 20 years with a mean of 9.7455 +- 4.5600 years. Age at onset of disease ranged from 1 to 19 years with a mean of 7.1308 +- 3.93107 years. Mean age at onset was early in male patients. Mean age at onset in male patients was 6.5757 +- 4.4749 years while in female patients it was 7.9274 +- 2.8948 years. Duration of the disease at presentation ranged from 2 months to 18 years with an average of 2.6271 +- 3.4410 years. The average duration of disease at first presentation was lesser in female patients as compared to male patients. The average duration of disease at first presentation was 1.3717 +- 1.5185 years in female patients and 3.5021 +- 4.1041 years in male patients.
The disease started during first five years of life in 28.6% of patients, between 5 to 10 years in 48.2% of patients and between 10 to 15 years in 21.4% of patients. The disease started in first decade in 44 (78.6%) patients. The onset was in second decade in 21.4% of patients (table-I). The disease started during first decade of life in 85% (n = 28,) of male patients and in 70% (n = 16) of female patients. The disease was more likely to start in first decade in male patients as compared to female patients. The most frequent age of onset of segmental vitiligo was found to be between 5 to 10 years (table-I). Family history of vitiligo was found in 6 (10.7%) patients of which 4 were male and 2 were female. The involvement of right and left sides of the body was almost equal in either sex. Left side involvement (30 patients: male, 18; female, 12) was almost the same as right side involvement (26 patients: male, 15; female, 11). The trigeminal dermatome was most commonly affected in either sex.
The disease most commonly involved the area of innervation of Maxillary division of Trigeminal nerve followed by thoracic, cervical, lumbar, and sacral dermatome in either sex, in descending order of frequency (table-II). Face was the most common site of involvement, regardless of sex, followed by lower limbs and trunk (table-II). 15 of the 26 patients (57.7%) with involvement of face were female. This preponderance of female patients amongst those with involvement of face was statistically significant (p<0.05). Koebner phenomenon was observed in one patient (1.78%). Associated diseases were present in 2 (3.57%) patients. One of these two had atopic eczema while the other had lichen planus which was found co localized to the dermatome affected by vitiligo.
Vitiligo is a multifactorial polygenic disorder characterized by patchy depigmentation which is often progressive2,3,13. Different intrinsic, metabolic and functional defects appear to affect melanocytes leading to a substantial loss of functioning epidermal, and sometimes hair follicle melanocytes2,12. The disease is currently classified into two major subtypes: Segmental vitiligo (type B), which usually does not cross the midline; and (2) and non-segmental vitiligo (type A), also simply called "vitiligo" without qualification1,3,14-16. Recently, mixed vitiligo (MV) has been described which has an initial segmental involvement which is associated in a second step usually after several months with bilateral NSV patches15. Segmental vitiligo is a unique subset of vitiligo which exhibits several different features from non-segmental vitiligo3-5,10,12-17. A number of studies have described the clinical profile of Segmental vitiligo12,14,16.
The clinical profile of segmental vitiligo in a group of Pakistani children has been described in this study. The prevalence of segmental vitiligo within a total vitiligo population varies amongst different studies. Handa et al7 reported 5.0%, Habib et al9 reported 6.5%, Khaitan et al 18 6.7%, Liu et al 7 9.99%, de Barros et al19 11.3%, Berti et al20 8.0%, Hann et al12 16.1%, Koga and Tango et al16 27.9%; and in this study the prevalence was 8.26%. The prevalence of segmental vitiligo is higher in childhood vitiligo8,10,17-21,22-24. Mean age at presentation of Segmental vitiligo has variously been described. Habib et al9 reported 16.8 years, Khaitan et al19 reported 16.23 years, Barona et al25 19.7 years, Liu et al7 15.55, Hann et al12 reported 20 years, Park et al26 reported 26.06 years and in this study mean age at presentation was found to be 9.7455 +- 4.5600 years. The mean onset age of Segmental vitiligo in children was reported as 6.63 years by Li et al10.
The mean age at onset of Segmental vitiligo was found to be 14.4 years by Habib et al9, 15.6 years by Hann et al12, 15.55 years by Liu et al7, 16.3 years by Barona et al25 and 18.5 years Park et al26. In this study mean age of onset of disease was found to be 7.1308 +- 3.93107 years which was early as compared to the previous studies9,11-12,17,26. Mean age at onset was earlier in male patients (6.5757 +- 4.4749 years) as compared to female patients (7.9274 +- 2.8948 years) but the difference was not statistically significant. The disease started in first decade in 44 (78.6%) patients and the most frequent age of onset of segmental vitiligo was found to be between 5 to 10 years of age (n = 7, 48.2% of patients). Previously Li et al10 reported most frequent age of onset between 4 and 8 years (42.4%) and Liu et al7 reported most frequent age of onset between 5-14 years (48.4%).
Hann et al12 reported disease onset in first decade in 41.3% of their patients. Koga and Tango16 also reported that segmental vitiligo occurs in young patients. Average duration of disease has been reported to be 2 years for Segmental Vitiligo by Li et al10, 2.79 years by Ezzedine et al11 11, 3.67 years by Park et al25, 4.2 years by Khaitan et al18, 4.8 years (male patients, 4.2 years; female, 5.3 years) by Hann et al12. In this study average duration of disease was 2.6271 +- 3.4410 years which was in concordance with previous studies. The average duration of disease at first presentation was lesser in female patients (1.3717 +- 1.5185 years) as compared to male patients (3.5021 +- 4.1041 years). Family History of vitiligo was found to be present in 26.7% of cases of Segmental vitiligo by Habib et al9. Barona et al25 reported family history of vitiligo in 9.5% of their patients. Hann et al12 found family history of vitiligo in 11.5% of their patients.
Khaitan et al18 reported family history of vitiligo in 13.2% of their patients. In this study family history of vitiligo was observed in 10.7% of patients which was in concordance with previous reports. The trigeminal dermatome was most commonly affected in either sex. The disease most commonly involved the area of innervation of Maxillary division of Trigeminal nerve followed by thoracic, cervical, lumbar, and sacral dermatome in either sex, in descending order of frequency. This was in concordance with previous studies10-12.
Face was the most common site of involvement (n = 26, 46.4%) regardless of sex, followed by lower limbs and trunk. The pre-dominant occurrence of disease on head and neck areas has been reported previously12,17,24,26 but an explanation for this predilection for head and neck areas has never been given. One possibility could be greater cosmetic awareness of the patients about more visible lesions or for that matter parents in case of children. This is further supported by preponderance of female patients amongst those with involvement of face. Associated diseases were found in 2 (3.57%) patients in this study. One of these two had atopic eczema while the other had lichen planus. Occurrence of autoimmune disease in patients with segmental vitiligo has been reported variously by previous studies. Previously Li et al10 reported associated diseases in 4.6% of their patients. Hann et al12 reported associated diseases in 6.7% of their patients.
Khaitan et al18 reported associated diseases in 1.06%, Park et al25 showed that about 9.5% of Segmental vitiligo cases were associated with other diseases while autoimmune diseases, in 11% of their patients. The frequency of Halo nevi in patients with Segmental vitiligo was reported to be 10% by van Geel et al27, 10.2% by Ezzedine et al11 and 6.4% by Barona et al24 and has been considered to be a marker for progression of segmental vitiligo to non-segmental vitiligo. Halo nevi were not found in any of the patients in this study. Previously Hu et al8 also did not find Halo nevi in any of their patients with Segmental vitiligo. Koebner phenomenon was reported in 4.4% of their patients by Barona et al25, in 5.3% of patients by Khaitan et al18 and Hann et al12. In this study Koebner phenomenon was observed in one patient (1.8%).
Segmental vitiligo often begins in childhood and often occurs on face. The disease is characterized by significantly lower rate of association with other autoimmune diseases.
LIMITATION OF STUDY
Sample size was relatively smaller for such kind of epidemiological studies.
CONFLICT OF INTEREST
This study has no conflict of interest to be declared by any author.
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|Publication:||Pakistan Armed Forces Medical Journal|
|Date:||Jun 30, 2019|
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