Childhood vitiligo differs from adult-onset vitiligo for several features including increased incidence of the segmental variant, higher prevalence of halo nevi, and more common family history for autoimmune diseases and atopic diathesis. The major differential diagnoses are the postinflammatory hypomelanoses for nonsegmental vitiligo and nevus depigmentosus for segmental vitiligo. From a therapeutic standpoint, early awareness of the diagnosis seems to correlate with a good treatment outcome in this age group.
Key points
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Childhood vitiligo differs from adult-onset vitiligo for several features including more segmental forms, higher prevalence of halo nevi, and more common family history for autoimmune diseases and atopic diathesis.
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The major differential diagnoses are the postinflammatory hypomelanoses for nonsegmental vitiligo and nevus depigmentosus for segmental vitiligo.
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From the therapeutic standpoint, early awareness of the diagnosis seems to correlate with a good treatment outcome in this age group.
Introduction, epidemiology, and classification
Childhood vitiligo differs from adult-onset vitiligo for several features, but is basically the same disease, with a potentially better regenerative capacity of the melanocytic lineage. The exact prevalence of vitiligo in the pediatric age group is unknown but the figure of approximately 25% of onset of vitiligo before the age of 10 years obtained in Denmark seems correct. The mean age of onset in pediatric series varied among different studies from 4 to 8 years, but very early onset, as young as 3 months, is acknowledged, whereas congenital vitiligo is usually piebaldism misdiagnosed as vitiligo. The existence of true congenital vitiligo remains controversial. In fair-skinned individuals, vitiligo patches are usually detected only after the first exposure of the skin to sunlight, following the first summer of life. Girls predominate in reported pediatric series, but population-based studies do not confirm a sex bias.
The most common form of vitiligo in children is the nonsegmental type or “vitiligo” based on the international consensus. Nevertheless, the percentage of segmental vitiligo (SV) is higher in children compared with adults, whatever the ethnic background, and suggests a mosaic skin developmental predisposition. The prevalence of SV in childhood varies from 4.6% to 32.5% in published reports (reviewed in Ref. ). Mixed vitiligo is a more recently described, mostly pediatric subtype, with segmental involvement preceding typical generalized vitiligo. This presentation may exist in adults but is probably more frequently masked by widespread bilateral lesions.
Introduction, epidemiology, and classification
Childhood vitiligo differs from adult-onset vitiligo for several features, but is basically the same disease, with a potentially better regenerative capacity of the melanocytic lineage. The exact prevalence of vitiligo in the pediatric age group is unknown but the figure of approximately 25% of onset of vitiligo before the age of 10 years obtained in Denmark seems correct. The mean age of onset in pediatric series varied among different studies from 4 to 8 years, but very early onset, as young as 3 months, is acknowledged, whereas congenital vitiligo is usually piebaldism misdiagnosed as vitiligo. The existence of true congenital vitiligo remains controversial. In fair-skinned individuals, vitiligo patches are usually detected only after the first exposure of the skin to sunlight, following the first summer of life. Girls predominate in reported pediatric series, but population-based studies do not confirm a sex bias.
The most common form of vitiligo in children is the nonsegmental type or “vitiligo” based on the international consensus. Nevertheless, the percentage of segmental vitiligo (SV) is higher in children compared with adults, whatever the ethnic background, and suggests a mosaic skin developmental predisposition. The prevalence of SV in childhood varies from 4.6% to 32.5% in published reports (reviewed in Ref. ). Mixed vitiligo is a more recently described, mostly pediatric subtype, with segmental involvement preceding typical generalized vitiligo. This presentation may exist in adults but is probably more frequently masked by widespread bilateral lesions.
Personal and familial predisposing factors
Pajvani and colleagues reported that among children with vitiligo, those with a family history of vitiligo were more likely to have an earlier age of onset of the disease than those with a negative family history. The incidence for autoimmunity found in families of childhood vitiligo patients across different reported studies ranges from 3.3% to 27.3%. Halder and colleagues showed that a family history of autoimmunity was more frequently reported in children with vitiligo, as compared with adults with vitiligo. Mazereeuw-Hautier and colleagues found a similar percentage of familial autoimmune diseases in children with SV and non-SV.
A so far neglected finding has been the association of vitiligo with atopic dermatitis, a common finding at vitiligo pediatric clinics, with either associated/ongoing atopic dermatitis or with a well-documented personal history of atopic dermatitis or other atopic manifestations. This association has been confirmed in larger series and simultaneously genome-wide association studies have shown predisposing genes common to atopy and vitiligo, such as thymic stromal lymphopoietin.
Clinical characteristics
Knees, elbows, shins, arms, and hands are often the sites of initial involvement in children. Such locations are frequently scraped and scratched in this age group, but it is not known if this phenomenon occurs more or less commonly than in adults. The recently developed K-VSCOR indicates a correlation between rapid spread and koebnerization.
At diagnosis, the most common location of SV is the face, followed by the trunk, neck, and limbs ( Fig. 1 ). The face is also the most common location of vitiligo, especially around the eyes and neck, followed by the lower limbs, trunk, neck, and upper limbs. A burning sensation of the eyelids is a clue to the onset of vitiligo in fair-skinned children during outdoor activities. Involvement of the perineum and in particular perianal and buttocks skin is a common onset location of vitiligo in toddlers, suggesting a role of the Koebner phenomenon triggered by nappies and hygiene care ( Fig. 2 ).
Leukotrichia in the scalp area, sometimes preceding the onset of vitiligo by several months or years, is noticed occasionally including probably some cases of halo nevi more difficult to detect in this location. Independent of interfollicular skin involvement, premature diffuse graying of the hair is sometimes observed, which may correspond to the recently delineated follicular vitiligo.
The presence of halo nevi, and leukotrichia, in a patient with SV may be a risk for mixed vitiligo. The significance of isolated halo nevi as a clinical marker of risk for vitiligo is frequently debated. A comparison with the general pediatric population is difficult because the prevalence of halo nevi is not well known, possibly around 1%. Prcic and colleagues found that there are more halo nevi in children with vitiligo, compared with children without vitiligo (34% vs 3.3%). The prevalence of halo nevi in children with vitiligo varies widely according to series, from 2.5% to 34% (reviewed in Ref. ), and based on personal experience some cases would have gone undetected without Wood lamp examination. It is also unclear whether the prevalence of halo nevi in children with vitiligo is different from that found in adult vitiligo, but in general they are more easy to detect, without a background of skin aging. Vitiligo may develop within or around congenital nevi, and unusually on Becker nevus ( Fig. 3 ).
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Skin Cancer Risk (Nonmelanoma Skin Cancers/Melanoma) in Vitiligo Patients
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