10.5826/dpc.1304S2a312S
Vitiligo is the most common cause of depigmentation and its estimated worldwide prevalence ranges from 0.5% to 2%. The disease is characterized by the development of white macules resulting from a loss of epidermal melanocytes. The term vitiligo (nonsegmental) is now a consensus umbrella term for all forms of generalized vitiligo. Two other subsets of vitiligo are segmental vitiligo and unclassified/undetermined vitiligo, which corresponds to focal disease and rare variants. A series of hypopigmented disorders may masquerade as vitiligo, and some of them need to be ruled out by specific procedures including a skin biopsy. The skin plays an important role in our interaction with the world and any change in the skin colour can have important psychological consequences. In this line, vitiligo has a major impact on quality of life.In this review, we will detail the most recent data on the clinical features of vitiligo and its impact on quality of life.
text/xml
2160-9381
3892
en
Seneschal Julien
https://creativecommons.org/licenses/by-nc/4.0
Dermatology Practical & Conceptual
mental health
Clinical Features of Vitiligo and Social Impact on Quality of Life
Text.Serial.Journal
https://dpcj.org/index.php/dpc
Review
2023-12-19
2023-09-26
2023-12-19
2024-01-25
e2023312S
Dermatol Pract Concept
Vitiligo is the most common cause of depigmentation and its estimated worldwide prevalence ranges from 0.5% to 2%. The disease is characterized by the development of white macules resulting from a loss of epidermal melanocytes. The term vitiligo (nonsegmental) is now a consensus umbrella term for all forms of generalized vitiligo. Two other subsets of vitiligo are segmental vitiligo and unclassified/undetermined vitiligo, which corresponds to focal disease and rare variants. A series of hypopigmented disorders may masquerade as vitiligo, and some of them need to be ruled out by specific procedures including a skin biopsy. The skin plays an important role in our interaction with the world and any change in the skin colour can have important psychological consequences. In this line, vitiligo has a major impact on quality of life.In this review, we will detail the most recent data on the clinical features of vitiligo and its impact on quality of life.
Seneschal Julien
2023/12/19
10.5826/dpc.1304S2a312S
e2023312S
Department of Dermatology and Pediatric Dermatology, National Reference Center for Rare Skin disorders, Hôpital Saint-André; University of Bordeaux, CNRS, Immuno ConcEpT, UMR 5164, Bordeaux, France 
2160-9381
mental health
en
e2023312S
Manzoni APD da S, Weber MB, Nagatomi AR da S, Pereira RL, Townsend RZ, Cestari TF. Assessing depression and anxiety in the caregivers of pediatric patients with chronic skin disorders*. An Bras Dermatol 2013;88:894–9.
Clinical Features of Vitiligo and Social Impact on Quality of Life
https://dpcj.org/index.php/dpc/article/download/3892/2436
                   Clinical Features of Vitiligo and Social
                          Impact on Quality of Life
                                                           Julien Seneschal1,2


1 Department of Dermatology and Pediatric Dermatology, National Reference Center for Rare Skin disorders, Hôpital Saint-André,
  Bordeaux, France
2 University of Bordeaux, CNRS, Immuno ConcEpT, UMR 5164, Bordeaux, France



Key words: vitiligo, mental health, social impact, quality of life
Citation: Seneschal J. Clinical Features of Vitiligo and Social Impact on Quality of Life. Dermatol Pract Concept.2023;13(4)S2:e2023312S.
DOI: https://doi.org/10.5826/dpc.1304S2a312S
Accepted: December 11, 2023; Published: December 2023
Copyright: ©2023 Seneschal. This is an open-access article distributed under the terms of the Creative Commons Attribution-
NonCommercial License (BY-NC-4.0), https://creativecommons.org/licenses/by-nc/4.0/, which permits unrestricted noncommercial use,
distribution, and reproduction in any medium, provided the original authors and source are credited.
Funding: None.
Competing Interests: Julien Seneschal: received grants and/or honoraria from AbbVie, Bristol Myers Squibb, Calypso Biotech, Eli Lilly,
Incyte, LEO Pharma, Novartis, Pfizer, Pierre Fabre, Sanofi, Sun Pharmaceuticals, and Viela Bio; and has a patent on MMP9 inhibitors and
uses thereof in the prevention or treatment of a depigmenting disorder and three-dimensional model of depigmenting disorder.
Corresponding Author: Julien Seneschal M.D, Ph.D., Department of Dermatology, Hôpital Saint-André, CHU de Bordeaux, Bordeaux,
France. Bordeaux University, CNRS, ImmunoConcept, UMR 5164, 33000 Bordeaux, France. Phone : +33(0)5 56 79 47 05 Fax : +33(0)5
56 79 49 75 E-mail : julien.seneschal@chu-bordeaux.fr




  ABSTRACT              Vitiligo is the most common cause of depigmentation and its estimated worldwide prevalence ranges
                        from 0.5% to 2%. The disease is characterized by the development of white macules resulting from a
                        loss of epidermal melanocytes. The term vitiligo (nonsegmental) is now a consensus umbrella term for
                        all forms of generalized vitiligo. Two other subsets of vitiligo are segmental vitiligo and unclassified/
                        undetermined vitiligo, which corresponds to focal disease and rare variants. A series of hypopigmented
                        disorders may masquerade as vitiligo, and some of them need to be ruled out by specific procedures
                        including a skin biopsy. The skin plays an important role in our interaction with the world and any
                        change in the skin colour can have important psychological consequences. In this line, vitiligo has a
                        major impact on quality of life.
                        In this review, we will detail the most recent data on the clinical features of vitiligo and its impact on
                        quality of life.




Introduction                                                          of occurrence according to phototype or race [1,2]. Twenty
                                                                      five percent of cases are children with disease onset before the
Vitiligo is the most common skin depigmenting disorder re-            age of 10, the age of onset in paediatric series varies from 4 to
sulting from a selective loss of epidermal melanocytes and af-        8 years. Very early onset, as young as 3 months, is acknowl-
fects around 0.5-2 % of the world population. Both sexes are          edged. The existence of true ‘congenital vitiligo’ remains con-
equally affected, and there are no apparent differences in rates      troversial [3,4]. In fair-skinned individuals, vitiligo patches



Review | Dermatol Pract Concept. 2023;13(4)S2:e2023312S                                                                                1
are usually detected only after the first exposure of the skin        a clinically unilateral segmental distribution of depigmented
to sunlight, following the first summer of life. The percentage       lesions. The coexistence of SV plus vitiligo (non-segmental) is
of segmental vitiligo (SV) is higher in children compared to          called mixed vitiligo. Focal vitiligo, a term that applies to lo-
adults, whatever the ethnic background, suggesting a mosaic           calized macules characterized by loss of melanocytes, was as-
skin developmental predisposition. The prevalence of SV in            signed to the category undetermined/unclassified until more
childhood varies from 4.6 to 32.5% in published reports. In           definitive classification can be made on clinical grounds
addition, besides the recognition of clinical lesions of vitiligo,    (generally after 1–2 years of follow-up).
it is now important to recognize clinical signs of disease that
could justify for patients with a progressive disease a treat-        Vitiligo Subsets
ment to stop the spreading of the disease.                            Generalized Vitiligo
    In addition, vitiligo is always associated with major im-         This most common form of vitiligo is characterized by white
pact on quality of life [5]. This review summarizes classifi-         macules involving multiple parts of the body, most often in
cation, clinical aspects, and current knowledge regarding the         a symmetrical pattern (Fig. 1). Skin hypopigmentation is
impact on quality of life of patients affected by vitiligo, a still   usually asymptomatic, but a minority of patients mention
high unmet disease.                                                   preceding mild pruritus (probably due to the inflammation).
                                                                      The disease can start at any site of the body, but the fingers,
Clinical Features of Vitiligo                                         hands, and face are frequently the initial sites. Depigmen-
Vitiligo is characterized by the progressive loss of melano-          tation of scars is a common manifestation of the Koebner’s
cytes leaving white patches on the skin. It is usually diag-          phenomenon (mechanical induction of the disease, also by
nosed by clinical examination alone supported by Wood’s               friction or chronic pressure by clothing or daily activities).
lamp examination. The differential diagnoses of vitiligo              Koebner’s phenomenon is usually contemporary of disease
include pityriasis alba, hypopigmented mycosis fungoides,             flares [6]. Stable lesions are well demarcated. Mixed vitiligo
tinea versicolor, idiopathic guttate hypomelanosis, and other         is a more recently described, mostly paediatric subtype, with
hypo- or depigmented disorders. Additionally, the diagno-             segmental involvement preceding typical generalized vitiligo
sis of vitiligo could be difficult in patients with fair skin. In     [7]. The presence of leukotrichia and halo nevi have been
cases of uncertain diagnosis, a skin biopsy, mycologic ex-            noted as predictors of passage to mixed vitiligo in patients
amination, and appropriate blood tests may be needed to               with SV [8]. Mixed vitiligo may exist in adults but is proba-
exclude a fungal infection, cutaneous lymphoma and other              bly frequently masked by widespread bilateral lesions.
disorders. Routine screening of anti-thyroid antibodies and
thyroid function are recommended. The term “vitiligo (non-­           Acrofacial Vitiligo
segmental)” ­referring to all forms of vitiligo except segmen-        In acrofacial vitiligo, the involved sites are usually limited
tal vitiligo is now proposed. Segmental vitiligo (SV) refers to       to face, head, hands, and feet (Fig 1). A distinctive feature




Figure 1. Clinical features of vitiligo



2                                                                             Review | Dermatol Pract Concept. 2023;13(4)S2:e2023312S
is depigmentation of the distal fingers and facial orifices. It     Clinical Signs of Disease Activity
may later include other body sites, resulting in typical gener-     Recently it has been defined different clinical signs associ-
alized vitiligo. Acrofacial vitiligo was shown to be more fre-      ated with the progression of the disease. Several visible clin-
quent in adult onset cases of vitiligo in a large series studies    ical skin manifestations in vitiligo are reported in relation to
using latent class analysis [9].                                    disease progression such as inflammatory borders, Koebner
                                                                    phenomenon, hypochromic areas/ borders and confetti-like
Vitiligo Universalis
                                                                    depigmentation [12]. The presence of these clinical markers
Vitiligo universalis is a rare presentation of vitiligo. It is      in patients with vitiligo has been linked to poor prognosis,
the most extensive form of the disease and generally occurs         rapid disease progression and inadequate response to ther-
in adulthood. ‘Universalis’ is generally used when depig-           apies. Confetti-like depigmentation was first described by
mentation is virtually universal (80–90% of body surface),          Sosa JJ and defined as the presence of several grouped 1 to
but some pigmentation may be still present, and hairs par-          5-mm depigmented macules, usually located at the border of
tially spared.                                                      an existing lesion [13]. Therefore, these signs are important
                                                                    to recognize to start as soon as possible treatment that could
Segmental Vitiligo
                                                                    stop the spread of the disease (Fig. 2).
Mono-segmental vitiligo is the most common form of SV,
referring to the presence of one or more white depigmented
macules distributed on one side of the body, usually respect-       Social Impact on Quality of Life
ing the midline (although some lesions may partly cross the         Skin plays an important role in our interaction with the
mid-line), early follicular involvement (leukotrichia), and         world, and skin colour is an important element in our in-
rapid development over a few weeks or months, and overall           teraction with the world. In this sense, any change in skin
protracted course but secondary extension remains possible          colour can have important psychological consequences. In
in a given segment sometimes years after [10,11]. The aetiol-       patients with vitiligo, quality of life and disease burden can
ogy of the SV pattern remains overall elusive. Rarely, multi-       be measured by generic dermatology instruments such as the
ple segmental lesions occur simultaneously or not distributed       Daily Life Quality Index (DLQI). Although generic instru-
either unilaterally or bilaterally. A clear segmental distribu-     ments such as the DLQI can provide a general picture of
tion of the lesions with midline demarcation, together with         impaired quality of life, they generally fail to detect nuances
the associated features described in mono-segmental cases           in the way patients manage the overall burden of vitiligo. In
(leukotrichia, protracted course), distinguishes this diagnosis     this context, over the last decade, several specific scores to
versus vitiligo in bilateral cases.                                 measure the individual impact of the disease have also been
                                                                    developed, such as the Vitiligo Impact Scale (VIS), the Vitil-
Unclassified and Rare Variants                                      igo Quality Of Life (VitiQoL) or the Vitiligo Impact Patient
Focal cutaneous or mucosal vitiligo (defined as small isolated      Scale (VIPs) [14]. Of all these scores, only the VIPs considers
patch that does not fit a segmental distribution, and which         the patient’s phototype, which is a major factor in the expe-
has not evolved into vitiligo after a period of at least 2 years)   rience of the disease [15]. In the late 1970s, Porter et al. first
should be left within the category undetermined⁄ unclassified       reported the major impact of vitiligo on patients’ quality of
vitiligo. Vitiligo ponctué/ punctata lesions present as sharply     life and there is now strong evidence that patients with vitil-
demarcated depigmented punctiform 1- to 1.5-mm macules              igo are negatively affected in their sexual relationships [16].
involving any area of the body, and has to be distinguished         In addition, several studies have shown that adult patients
histopathologically from guttate hypomelanosis, a common            with generalized vitiligo experience a decrease in quality of
condition with no loss of melanocytes situated on chronically       life comparable to that of patients with other skin diseases
sun exposed sites such as the legs and forearms. Vitiligo minor/    such as atopic eczema and psoriasis [5]. Indeed, many people
hypochromic vitiligo to affect only dark-skinned individu-          are afraid and uncomfortable when they come into contact
als. ‘Minor’ refers to a partial defect in pigmentation. The        with people with vitiligo, thus discriminating against these
relation to true vitiligo comes from pathology and coexis-          patients. Vitiligo patients also complain that they do not re-
tence with more typical vitiligo macules. Cutaneous T cell          ceive enough support from their doctors. More recently, in
lymphoma needs to be ruled out by repeated biopsies with            2005, a survey of members of the United Kingdom Vitiligo
molecular studies of clonality, and this diagnosis cannot be        Society showed that over 50% of respondents said that vit-
made without a long-term follow-up. Follicular Vitiligo re-         iligo had a significant impact on their quality of life. In this
fers to a form of generalized vitiligo that primarily involved      study, finding an effective and long-lasting treatment was the
the pigment cell follicular reservoir with limited skin involve-    top priority for the patients who responded to the survey
ment, contrasting with marked generalized hair whitening.           and who had the most severe forms. Only a small number of



Review | Dermatol Pract Concept. 2023;13(4)S2:e2023312S                                                                             3
Figure 2. Clinical signs of disease activity. a. clinical images shows the presence of confettis-like lesions and koebner phenomenon type 2b
                                                                                                                                          ​​​​​​​
(linear lesion on site of clinical friction). b. evolution of vitiligo without effective treatments in patients with active disease




respondents reported getting information about vitiligo from              that people with vitiligo suffer from low self-esteem, social
their dermatologist (12.5%), while more than 80% get this                 stigma, shame, avoidance of intimacy, anxiety, depression,
information from non-medical sources [17]. More generally,                adjustment disorders, fear, suicidal ideation and other psy-
several factors can have an impact on the quality of life of              chiatric comorbidities [24]. In an internet survey of children
vitiligo patients, namely age of onset, skin phototype, extent            aged 0- 17 years and their families, the deterioration in qual-
and distribution, impact of treatment, among others. For ex-              ity of life increased with age: more than 90% of teenagers
ample, Nogueira et al. found that most vitiligo patients with             (15-17 years) were bothered by vitiligo compared to 50% of
affected areas corresponding to visible areas (88%) com-                  children aged 0-14 years [22]. The localization of the disease
plain of negative emotions compared to those with lesions                 vitiligo on the face, arms and legs was found to be the most
in non-visible areas (20%). Illness (71%), shame (57%), in-               distressing and was associated with teasing and bullying. Fi-
security (55%), sadness (55%) and inhibition (53%) were                   nally, depression and anxiety in children were reported by
the most frequently cited negative emotions in patients with              26% and 42% of parents and caregivers respectively [25].
visible lesions [18,19]. Similarly, Parsad et al. found that in-
juries to the face, arms, legs and hands had a greater impact
on DLQI [20]. The degree of stigma is probably related to                 Conclusion
cultural background and may therefore explain some of the
                                                                          Vitiligo is a common disease with an estimated global prev-
variation in DLQI observed between countries [21]. Spe-
                                                                          alence of 1%. Its impact on quality of life and self-esteem is
cifically, the self-image of vitiligo patients is significantly
                                                                          considerable and long-lasting, with possible repercussions in
impaired and mood insensitivity is common, especially in ad-
                                                                          adulthood for children with the disease. In this context, it is im-
olescents [22]. The psychological impact of vitiligo in child-
                                                                          portant to listen to patients and to take charge of their psycho-
hood can be profound and have a lasting effect on personal
                                                                          logical suffering by not hesitating to offer psychological care.
self-esteem. Parsad et al. found that children with vitiligo
were more likely to avoid/ restrict sports activities and miss
more school days than those without vitiligo [20]. In another             References
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Review | Dermatol Pract Concept. 2023;13(4)S2:e2023312S                                                                                         5
