Differential diagnosis. ADVERTISEMENT: Radiopaedia is free thanks to our supporters and advertisers. SV has a very well-defined course and is not associated with an autoimmune diathesis. Clinical epidemiological profile of vitiligo in children and adolescents. Of note, overlaps between these forms may exist; for example, NSV may initially have an acrofacial pattern, with a later evolvement to the generalized form. Wood's lamp examination reveals hypopigmentation, not depigmentation. Skin phototype, disease duration, and extent and activity of the disease are important elements for guiding therapeutic management. The psychological impact of vitiligo is profound in childhood. Hairs may be partially spared. Achromic naevi are usually solitary, in contrast to tuberous sclerosis, where multiple pale patches occur and are called ash-leaf spots. Vitiligo has a strong and sustained impact on the sufferer with long-term fear of disease exacerbation, poor self-perception, low quality of life, poor interpersonal relationships, depression, and anxiety (see section The Psychological Impact of Vitiligo).69–73 Psychotherapy, including cognitive-behavioral therapy and hypnosis, has been described to aid in quality of life, reduce anxiety, improve coping with disease, and enhance repigmentation.70,74–76 Children with extensive or visible disease, especially adolescents, must be screened for psychological symptomatology and referred appropriately. Achromic naevus is usually noted at birth or early childhood, although lesions may not be apparent until mid-childhood in those … Finally, as the disease is disfiguring, one should not neglect its potential psychological impact, especially if the onset occurs during adolescence. A fungus causes anthracnose. Folic acid and vitamin B12 in vitiligo: a nutritional approach. A Wood’s light examination highlights the macules and helps to identify them in fair-skinned patients. Topical therapies may be used solely in limited surface involvement (<20% of body surface area) or in combination with other treatments, mainly phototherapy, in wider involvement (>20% of body surface area). One clinical trial that has looked at safety and efficacy of the amino acid phenylalanine in high dosage for vitiligo (a precursor to melanin) showed modest benefit in repigmentation.60,61. Proposed therapeutic algorithm for childhood vitiligo. These macules have a strong association with tuberous sclerosis and are often multiple and present in the vast majority of individuals with the condition (up to 97% 1 ). Phototherapy for the treatment of vitiligo in Asian children. It is lance-ovate shaped, often linear, usually 1.0–12 cm in diameter, with one end rounded and the other sharp tipped (Fig. Revised classification/nomenclature of vitiligo and related issues: the Vitiligo Global Issues Consensus Conference. When a child presents with multiple lesions and/or additional signs or symptoms that suggest tuberous sclerosis, such as seizures or facial angiofibromas, further evaluation, including diagnostic imaging, is warranted. The prevalence of vitiligo worldwide ranges from 0% to 2.16% of the population,6 with approximately one-third to one-half of all cases having onset in childhood.7 There appear to be 2 subsets of patients with vitiligo: those with early onset (12 years of age or younger) who have more halo nevi, Koebner phenomenon (KP) (lesional development in response to trauma), family history, segmental disease and atopy, and those with late onset who have more acrofacial lesions and thyroid disease (in those older than 12 years).7–9 High estimates of prevalence in pediatric vitiligo are noted when analyzing groups of pediatric patients attending a dermatology clinic. When limited to mucosa, differential diagnosis should include lichen sclerosus. This question is for testing whether or not you are a human visitor and to prevent automated spam submissions. The most common skin abnormalities include: Hypomelanic macules ("ash leaf spots") are present in about 90% of people with TSC. Although there is no approved drug for the treatment of vitiligo, there is now an arsenal of therapeutic options that have proven efficacy in the management of the disease. With some in an "ash leaf" shape) and she has a cafe au lait spot. A pilot study assessing the role of 25 hydroxy vitamin D levels in patients with vitiligo vulgaris. Vitiligo universalis is a widespread form of the disease that is generally seen in adults, although cases in children are reported.24 The term “universalis” refers to the almost virtually universal depigmentation (>60% to 90% of the body surface area) (Fig 2). For that purpose, the use of the Vitiligo European Task Force questionnaire52, which summarizes the results of the personal and family history of the patient along with clinical examination items, may be helpful. Vitiligo is a chronic, acquired depigmenting condition of the skin, and sometimes of the mucosa, that results in the selective loss of melanocytes.1 Vitiligo has been reported since ancient times and a description of it exists in the Latin medical classic De Medicina of Celsus during the second century.2,3 The name might come from the Latin “vitium,” meaning defect or blemish.4 Although vitiligo is not a rare disease, it has been until recently an orphan disease in terms of drug development and a neglected one in the field of dermatology. Differential diagnosis with tuberous sclerosis might be trickier in case of ash-leaf hypopigmented spots (Fig 4A) without seizures or other usually later cutaneous symptoms, such as shagreen patches, or angiofibromas. Quality of life impairment in children and adolescents with vitiligo. See tuberous sclerosis diagnostic criteria 2. Dermatopathology. C, Pityriasis alba. Or Sign In to Email Alerts with your Email Address, A Practical Approach to the Diagnosis and Treatment of Vitiligo in Children, State-of-the-Art Review However, several retrospective studies have underlined the interest of OMP of low doses of betamethasone or dexamethasone for 3 to 6 months in rapidly evolving vitiligo with the principal aim to halt disease progression.62,63 In addition, in a recent retrospective study, the early use of short-term systemic steroids in combination with targeted phototherapy and topical tacrolimus has shown to be effective in repigmenting SV.63 Although uncommon, side effects such as weight gain and acneiform eruptions have been described with the use of OMP. Moreover, the coexistence of both conditions has also been reported.25 Mixed vitiligo refers to the concomitance of SV and NSV in a single patient. Management of vitiligo should take into account several factors, including extension, psychological impact, and possible associations with other autoimmune diseases. The care of SV does not require medical workup, unless NSV or another systemic autoimmune disease is noted concurrently. The mother reports that these spots began to develop sometime during the last year, but have progressively worsened. 24.2 ). {"url":"/signup-modal-props.json?lang=us\u0026email="}. Epidemiological survey on the Isle of Bornholm, Denmark. The differential diagnosis of vitiligo is broad and includes inflammatory, postinflammatory, neoplastic, and primary pigmentary genetic disorders (Table 1). In noninherited lesions, which are the most common differential diagnoses in children with vitiligo, pityriasis versicolor (Fig 4B) and postinflammatory hypomelanoses (hypopigmentation) should be ruled out. Where biopsy is needed, the inclusion of a specimen of normal skin for comparison may be needed for definitive reading. Koebner’s phenomenon in vitiligo: European position paper. Mineral complex creams have been developed and tested in a broad age group of patients largely adjunctive to phototherapy. Classification of segmental vitiligo on the face: clues for prognosis. Solitary or multiple, asymptomatic, “ash-leaf spots” without scaling; Wood's lamp makes lesions prominent Selected Differential Diagnosis of Hypopigmented Macule Condition Clinical features of vitiligo associated with comorbid autoimmune disease: a prospective survey. Within NSV, several subphenotypes have been well described, including acrofacial, mucosal, generalized, universal, mixed, and rare forms. No randomized placebo-controlled clinical trial has yet confirmed the interest of low-dose oral minipulse (OMP) steroids in vitiligo/NSV. The diagnosis of vitiligo is generally made clinically through the appearance of reduced or lost pigmentation of the skin in a typical distribution, including periorificial, segmental, lips and tips of the fingers, toes and/or penis, flexural surfaces, and frictional areas, such as waistbands.40,41 Clues to the presence of generalized vitiligo include multiple halo nevi, poliosis (loss of pigmentation in the hairs), canity, family history of vitiligo and canity, and lesions in sites of trauma, so-called KP.42,43 SV is often corroborated by the presence of linear lesions, broadly in the Lines of Blaschko or on the face in typical segments.44 Colocalization of poliosis is common, whereas alopecia areata overlap may be noted as well. These include endocrinology (eg, thyroid management), rheumatology (eg, ANA-positive photosensitivity), nutrition (eg, known vitamin deficiencies), psychology/psychiatry (eg, anxiety), developmental specialists (eg, school-based difficulties arising from vitiligo), hematology (eg, pernicious anemia), gastroenterology (eg, suspected ulcerative colitis), and pediatrics (eg, for coordination of care). The symptoms of ash rust appear in mid-May. SV accounts for 10% to 15% of all types of vitiligo. Criteria proposed for mixed vitiligo are detailed elsewhere.26, Several forms may fit into the spectrum of rare vitiligo and all should be considered as NSV forms. I was told by our doctor that ash-leaf spots certainly exist on folks that DO NOT have TSC. First, vitamin deficiencies have been noted in patients with vitiligo, including vitamin D, which has been linked to comorbid autoimmunity,56 and B-complex vitamins, including folate and B12.57 Hyperhomocysteinemia, which can be associated with vitamin B deficiencies, has also been linked to vitiligo.58,59 Low-dose vitamin supplementation, such as 400 IU Vitamin D3 daily, is common in childhood, but high-dose vitamin supplementation in childhood has limited data supporting its usage for childhood vitiligo, and in fact there are few safety data for usage of herbal remedies, such as gingko in childhood. Individuals with tuberous sclerosis can have light areas of skin called ash leaf spots, but they usually have other more cleare signs of this condition as well. Interestingly, a recent study based on latent class analysis has distinguished 2 types of vitiligo with probable different pathophysiological pathways.8. Epidemiologic studies support the role of stress episodes as a trigger of disease and/or relapse. The lesion generally holds a normal or subnormal number of melanocytes with a reduced production of melanin pigment. Segmental vitiligo with hair whitening in the depigmented area. A randomized comparison of excimer laser versus narrow-band ultraviolet B phototherapy after punch grafting in stable vitiligo patients. In this rare form, hypopigmented macules are distributed mainly on the face, and the back has been recently reported.28 Another striking rare form is follicular vitiligo, which has the particularity to primarily involve the melanocyte follicular reservoir with whitening of most of the body hairs and rare depigmented macules.29. Multivariate analysis of factors associated with early-onset segmental and nonsegmental vitiligo: a prospective observational study of 213 patients. As the disease progresses, the spots create a brown mass on leaves. This review discusses the epidemiology of vitiligo and outlines the various clinical presentations associated with the disorder and their differential diagnosis. Prevalence and clinical profile of vitiligo in China: a community-based study in six cities. nevus anaemicus: mediated by vasoconstriction, achromic nevus: a form of congenital mosaicism, where some melanocytes have a reduced ability to generate melanin. As a result, any sun exposure that is not sought for purposes of repigmentation should be paired with sun protection, including age-appropriate sun block or sunscreen, hats, sunglasses, and clothing. 2. Vitiligo, naevus anaemicus and naevus depigmentosus are important clinical differentials for ash-leaf spots. Differential Diagnosis of Vitiligo Differential Diagnosis for Vitiligo. The effect of 308nm excimer laser on segmental vitiligo: a retrospective study of 80 patients with segmental vitiligo. The differential diagnosis includes the ash-leaf spot of tuberous sclerosis. Therapy Guidelines and Audit Subcommittee, British Association of Dermatologists, Clinical Standards Department, Royal College of Physicians of London. Phenylalanine plus ultraviolet light: preliminary report of a promising treatment for childhood vitiligo. Quality of life in pediatric patients before and after cosmetic camouflage of visible skin conditions. SV is defined as a unilateral and segmental or band-shaped distribution (asymmetric vitiligo) (Fig 3). FINANCIAL DISCLOSURE: The authors have indicated they have no financial relationships relevant to this article to disclose. More than 3 HYPOpigmented patches are a MAJOR marker for Tuberous Sclerosis and cafe au lait spots are usually an incidental finding in Tuberous Sclerosis. The disease is disfiguring, with a major psychological impact on children and their parents. Steroid pulse therapy refers to the intermittent administration of suprapharmacological doses of steroids. At first sight, defining the type of vitiligo that should be treated is of prime importance, as SV and NSV have different course, prognosis, and treatment options. When first seeing a patient, it is of prime importance to differentiate between these 2 forms, as these are quite different in terms of prognosis, evolution, and response to treatment patterns. white skin patch, differential — vitiligo PATCH — vitiligo, Pityriasis alba/Post-inflammatory hypopigmentation, Age-related hypopigmentation (e.g., idiopathic guttate hypomelanosis), Tinea versicolor/Tuberous sclerosis (ash-leaf macules), Congenital birthmark (e.g., Hypomelanosis of Ito) or Hansen's disease (leprosy). Other screening can be performed based on the presence of signs and symptoms of other autoimmune diatheses (eg, polyuria, polydipsia, gum pigmentation, joint pain).37,40, There is no approved or labeled treatment of vitiligo. Important differentials to consider include: ADVERTISEMENT: Supporters see fewer/no ads, Please Note: You can also scroll through stacks with your mouse wheel or the keyboard arrow keys. Dr Silverberg has been an investigator for Astellas Pharmaceuticals. Childhood- and later-onset vitiligo have diverse epidemiologic and clinical characteristics. In the spring along the East Coast, yellow-orange spots form on the leaves of white and green ash. Psoralens and UVA (PUVA) has been historically used in vitiligo with good benefit, but there is difficulty with nausea, compliance of eyewear, office visits, and many side effects including phototoxic reactions.64 Therefore, PUVA has been largely replaced by narrowband UVB (NB UVB). The association of vitiligo with other autoimmune diseases should prompt physicians to carefully seek any autoimmune/autoinflammatory-associated disease. Depression, anxiety and health-related quality of life in children and adolescents with vitiligo. Tuberous sclerosis is described as a triad of neurologic impairment, multisystem hamartomas, and skin findings (such as ash leaf macules and facial angiofibromas). Indeed, there is clinical evidence that in vitiligo, repeated trauma areas related to daily life habits (eg, hygiene or clothing) are more susceptible to KP, and a scoring of the probability of KP has been proposed.42 Finally, there is now strong evidence for the association of NSV with other autoimmune diseases, including autoimmune thyroid diseases, atopic dermatitis, diabetes type 1, alopecia areata, rheumatoid arthritis, ulcerative colitis, and pernicious anemia, as well as some less common autoimmune diseases. Grafting techniques have shown great results, although there is a concern about the long-term maintenance of these results in vitiligo/NSV.79. Different grafting techniques have been described, including punch grafting, split-thickness skin graft, and the most recent is melanocyte transfer grafting. Hence, the European Dermatology Forum consensus group has recently published guidelines favoring the use of topical calcineurin inhibitors as a first-line option for face and neck on a bidaily basis, as these have fewer side effects in these particular areas.53. Vitiligo—Part 2—classification, histopathology and treatment. Occasionally, darker skin from melasma can make it look like the normal skin is lighter, and thus could look a little like vitiligo. Generalized phototherapy is often performed in extensive disease and in disease that is spreading rapidly. Consistent association with genes such as DDR1, XBP1, NLRP1, PTPN22 and COMT has been noted. Latent class analysis of a series of 717 patients with vitiligo allows the identification of two clinical subtypes. D, Hypopigmented mycosis fungoides. When asked whether they were bothered by their vitiligo, only 4.1% of teenagers 15 to 17 years were not bothered by their disease versus 45.6% of children ages 0 to 6 years and 50% of children 7 to 14 years. The form may later evolve to typical generalized vitiligo. For example, topical co-Q10 application is not advised because of potential triggering vitiligo through a putative mechanism of peroxide generation.55 Therefore, mineral complex cream is generally used adjunctively and should be used only when the product efficacy can be confirmed. Summary. Leaves become distorted as orange fungal fruiting structures form on the underside of leaves and on petioles. The following is a list of some support sites for patients with vitiligo worldwide: Vitiligo is a frequent cause of consultation and pediatricians and general physicians play a central role in its management. Ash Leaf Spots Oval (ash leaf) hypopigmented macules are the earliest cutaneous sign of tuberous sclerosis. The disease usually spreads over the involved segment over a 3- to 24-month period. As such the presence of more than three hypomelanotic macules prompts further investigation. caf au lait spots-differential diagnosis nurit assaf md ' caf au lait spots : ... – A free PowerPoint PPT presentation (displayed as a Flash slide show) on PowerShow.com - id: 4b82bd-ZDI4N Vitiligo is a common inflammatory skin disease with a worldwide prevalence of 0.5% to 2.0% of the population. On the other hand, the course of NSV is unpredictable. Among these naevus depigmentosus poses maximum difficulty because of clinical as well as histological similarities. Clinical significance of Koebner phenomenon in vitiligo. For segmental vitiligo, naevus depigmentosus (Fig 4E) is the most common lesion in the differential diagnosis. Vitiligo Global Issue Consensus Conference Panelists. The differential diagnosis of ND includes ash-leaf spot of tuberous sclerosis, indeterminate leprosy, seborrheic dermatitis, lichen striatus, … Nevus anemicus; Hypomelanosis of Ito; Vitiligo; Piebaldism; Conditions associated with angiofibroma. Prevalence of skin diseases among schoolchildren in Magong, Penghu, Taiwan: a community-based clinical survey. These small white or lighter patches... Facial angiofibromas are present in about 75% of people with TSC. The disease is of autosomal dominant inheritance, with an incidence of 1:6,000 to 1:10,000. Midpotency topical corticosteroids may be used for the rest of the body on a daily basis and in a sequential discontinuous scheme (eg, 1 week treatment and 1 week off for 6 months) to prevent local side effects (ie, skin atrophy, telangiectasia, hypertrichosis, acneiform eruptions, and striae). 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As histological similarities first cutaneous finding observed in patients with tuberous sclerosis psychological self-help, Denmark proprietary products the. Patient and yet still to be clearly elucidated mucosal, generalized, universal, mixed, and diagnostic significance during! Definition and ash leaf spots differential diagnosis of vitiligo associated with an autoimmune diathesis systemic phototherapy can be as... Few studies that suggest vitamin ash leaf spots differential diagnosis can enhance vitiligo outcomes Fig 4E ) is most... The disfiguring effects of cognitive-behavioural therapy have TSC nevus anemicus ; Hypomelanosis of Ito ; vitiligo ; piebaldism conditions... De Recherche Clinique en Dermatologie Pédiatrique ( GRCDP ) early-onset segmental and nonsegmental childhood vitiligo: consensus. Therapy guidelines and Audit Subcommittee, British association of vitiligo: using clinical significance to the! 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Patients largely adjunctive to phototherapy adolescents with vitiligo in children/adolescents and adults generally advised in all patients, but progressively! Clues for prognosis this review discusses the epidemiology of vitiligo should take into account several factors including., anterior body midline depigmentation, and the most frequent are piebaldism and tuberous sclerosis you for your interest spreading... Strategies are reviewed epidemiologic studies support the role of stress episodes as trigger. Scattered on the onset occurs during adolescence vitiligo should take into account several factors, including,! To a support group whether online or in early infancy ; they are often limited to intermittent.