Vitiligo has a variety of presentations, including focal, acrofacial, segmental, and generalized forms. Thorough knowledge of these presentations is important to make the correct diagnosis. Signs of activity are important to recognize so that treatment is optimized. Clinical findings of confettilike depigmentation, trichrome and inflammatory vitiligo, and the Koebner phenomenon should alert the clinician that a patient’s disease is likely to worsen. These patients may require systemic treatment to stabilize their disease. Many other skin disorders present with hypopigmentation or depigmentation and must be distinguished to determine the right diagnosis, advise the patient on prognosis, and prescribe the correct treatment.
Key points
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Thorough knowledge of the various presentations of vitiligo is important to make the correct diagnosis.
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Signs of activity in vitiligo patients are important to recognize so that treatment selection is optimized.
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Many skin disorders present with hypopigmentation or depigmentation and must be distinguished from vitiligo to make the correct diagnosis and prescribe the right treatment.
Presentations of vitiligo
Vitiligo most commonly presents as bilaterally symmetric macules of depigmentation, with a preference for periorificial skin as well as skin prone to trauma. Frequent sites of presentation are the eyelids, nostrils, perioral skin, ears, axillae, elbows, wrists, hand, fingers, areolae, periumbilical skin, inguinal folds, genitals, knees, ankles, feet, and toes.
Generalized Vitiligo
Focal vitiligo
Focal vitiligo is a localized, small depigmented lesion that is isolated, lacks a segmental distribution and has not progressed into generalized vitiligo after 2 years. Sometimes there will be 2 to 3 lesions in 1 area, but if they start to form a linear arrangement it is more likely to be segmental vitiligo. Because vitiligo usually spreads over time, it is important to rule out other diseases in patients with focal vitiligo, such as nevus depigmentosus and trauma-induced leukoderma.
Segmental vitiligo
A unilateral patch of depigmentation is known as segmental vitiligo. This form, comprising 5% to 30% of all cases of vitiligo, does not cross the midline and is often linear or blocklike in shape. Segmental vitiligo usually presents in childhood, with 1 large study reporting a mean age of onset of 15.6 years. Areas of involvement are the face, trunk, neck, extremities, and scalp, in descending order of frequency. Lesions usually progress rapidly over 6 months to 2 years and then stabilize. Rarely, patients develop segmental vitiligo first and then present with generalized vitiligo, which is then called “mixed vitiligo.”
Acrofacial vitiligo
Many patients have bilateral, depigmented macules limited to the head and distal extremities. There are too many lesions to make a diagnosis of focal vitiligo but fewer than is typically seen in generalized vitiligo. The term “acrofacial vitiligo” has been used to describe this variant. Although many patients never develop truncal lesions, this form may progress to generalized vitiligo over time. Another variant, known as lip-tip vitiligo, only affects the lips, fingers, and toes, and has been described most often in South Asians.
Presentations of vitiligo
Vitiligo most commonly presents as bilaterally symmetric macules of depigmentation, with a preference for periorificial skin as well as skin prone to trauma. Frequent sites of presentation are the eyelids, nostrils, perioral skin, ears, axillae, elbows, wrists, hand, fingers, areolae, periumbilical skin, inguinal folds, genitals, knees, ankles, feet, and toes.
Generalized Vitiligo
Focal vitiligo
Focal vitiligo is a localized, small depigmented lesion that is isolated, lacks a segmental distribution and has not progressed into generalized vitiligo after 2 years. Sometimes there will be 2 to 3 lesions in 1 area, but if they start to form a linear arrangement it is more likely to be segmental vitiligo. Because vitiligo usually spreads over time, it is important to rule out other diseases in patients with focal vitiligo, such as nevus depigmentosus and trauma-induced leukoderma.
Segmental vitiligo
A unilateral patch of depigmentation is known as segmental vitiligo. This form, comprising 5% to 30% of all cases of vitiligo, does not cross the midline and is often linear or blocklike in shape. Segmental vitiligo usually presents in childhood, with 1 large study reporting a mean age of onset of 15.6 years. Areas of involvement are the face, trunk, neck, extremities, and scalp, in descending order of frequency. Lesions usually progress rapidly over 6 months to 2 years and then stabilize. Rarely, patients develop segmental vitiligo first and then present with generalized vitiligo, which is then called “mixed vitiligo.”
Acrofacial vitiligo
Many patients have bilateral, depigmented macules limited to the head and distal extremities. There are too many lesions to make a diagnosis of focal vitiligo but fewer than is typically seen in generalized vitiligo. The term “acrofacial vitiligo” has been used to describe this variant. Although many patients never develop truncal lesions, this form may progress to generalized vitiligo over time. Another variant, known as lip-tip vitiligo, only affects the lips, fingers, and toes, and has been described most often in South Asians.
Signs of activity
Determination of disease activity is important to assess prognosis and select the right treatment. In addition, establishing disease stability is critical when treating patients with surgical therapy, such as noncultured epidermal suspension grafting. The lack of erythema, scale, itching, burning or any other symptom in most patients with vitiligo causes difficulty in determining activity. However, a few clinical findings have been reported to be associated with disease activity and should be looked for during examination of a patient with vitiligo.
Koebner Phenomenon
Also known as an “isomorphic response,” the Koebner phenomenon is defined as the development of lesions at the site of traumatized, uninvolved skin. This finding is present in about one-third of patients by history. Those with greater body surface involvement and earlier age of involvement have a higher risk of the Koebner phenomenon. Careful examination for linear macules of depigmentation at sites of scratches, abrasions, and other traumatic occurrences is necessary in all patients with vitiligo to detect this important sign of activity.
Trichrome Lesions
As a lesion of vitiligo progresses, the depigmentation gradually expands, with a sharp line of demarcation between pigmented and depigmented skin. In patients with rapidly progressing vitiligo, however, there is often an intervening zone of hypopigmented skin, known as trichrome vitiligo ( Fig. 1 ). A biopsy of the border of these lesions reveals an inflammatory infiltrate and degeneration of the basal layer, which are signs of activity.
Inflammatory Vitiligo
A rare form of vitiligo, known as inflammatory vitiligo, presents as erythema on the areas of depigmentation and/or the border of the lesion. These patients usually give a history of rapid enlargement of their vitiligo lesions. Mild scale may be seen overlying the lesions and pruritus of the affected may areas be a presenting complaint. An infiltrate of lymphocytes and macrophages with concomitant disappearance of melanocytes has been reported in this form of vitiligo.
Confettilike Lesions
Confettilike macules of depigmentation have been recently reported as a marker of rapidly progressive vitiligo. These patients present with clusters of small, 1- to 5-mm macules, often at the periphery of existing lesions. The small lesions enlarge, and rapidly coalesce and form larger, more typical lesions of vitiligo. Affected patients have a higher Vitiligo Disease Activity Score and Koebner Phenomenon Vitiligo Score, which correlates with disease progression. Biopsy of confetti lesions reveals an inflammatory infiltrate of lymphocytes with CD8 + lymphocytes at the basal cell layer, where melanocytes reside.
Recognition of the signs of vitiligo activity is helpful in selecting therapy. Stable disease can usually be treated with topicals and phototherapy, which may take several months to achieve the first signs of repigmentation. However, patients with active disease may also need to be treated with oral corticosteroids for a period of time to stabilize their disease.
Differential diagnosis of vitiligo
Many disorders can mimic vitiligo, especially early vitiligo. A useful clinical approach is to classify them based on the extent of the lesions (localized or widespread), the pattern of lesions (eg, guttate/confetti) and the degree of pigment loss (depigmented or hypopigmented). Further clinical differentiation can be made on the basis of associated morphologic signs, including secondary changes of the epidermis (such as scaling and atrophy) and dermis (such as induration and infiltration; Figs. 2 and 3 ).
To differentiate between depigmented and hypopigmented lesions, Wood’s light is a useful bedside tool. Wood’s light is a form of long-wave ultraviolet radiation (320–400 nm, peak at 365 nm) emitted from a high-pressured mercury arc lamp after passing through a compound filter of barium silicate and 9% nickel oxide. In depigmented lesions, the absence of melanocytes results in a lack of absorption of Wood’s light by epidermal melanin causing most of the light to be reflected. A window is also created for autofluorescence of dermal collagen, giving off a small band of blue light. Depigmented lesions therefore appear as bright bluish-white patches under Wood’s light. This tool is useful in distinguishing established vitiligo (depigmented) in a dark room from hypopigmented conditions such as naevus anemicus, naevus depigmentosus, pityriasis alba, and postinflammatory hypopigmentation, which fail to accentuate. Wood’s light is also useful in evaluating vitiligo in fair-skinned individuals, where the lesion and its margins may be difficult to discern.
Localized Depigmentation
Many acquired conditions other than vitiligo can present with depigmentation. Depigmented lesions are porcelain white in color because of the complete absence of melanocytes; however, they can be admixed with hypopigmented lesions if the degree of pigment loss is variable. A careful history is of paramount importance in eliciting the precipitating factors and identifying the etiology. The acquired loss of melanocytes and reduction of melanin production can be owing to a variety of mechanisms, including infective, inflammatory, immune, and chemical- and drug-induced causes.
Focal depigmented lesions can occur in Treponemal diseases such as secondary syphilis (leucoderma syphiliticum) and late stage pinta. Although rarely seen, depigmented and hypopigmented macules and patches can present in patients with secondary syphilis. Classically described by Hardy in 1854 as leucodermic macules on the neck (necklace of Venus), these lesions can manifest on different parts of the body including the groin and genitalia. Histologic differentiation from vitiligo can be made by detecting lichenoid interface dermatitis, plasma cell infiltrates and endothelial edema. Serologic tests for syphilis are confirmatory. It remains controversial if the loss of pigment is a result of postinflammatory changes or a direct effect of spirochetes on melanocytes, because ultrastructural evidence of Treponema pallidum has been demonstrated within the leucodermic lesions. Unlike syphilis, pinta is predominantly found in Central and South America and is caused by T carateum . The disease is limited only to the skin, without systemic involvement. In late pinta, depigmented lesions have been classically described to appear symmetrically on bony prominences, such as the wrists, elbows, and ankles. Late leucodermic lesions of pinta are not infectious, unlike early pinta, which are teeming with treponemes.
Postinflammatory depigmentation is a particular problem in dark-skinned individuals. The diagnosis is straightforward when the primary lesion and its secondary changes are present. However, it can pose a diagnostic challenge if the preceding inflammatory changes have receded. Depigmentation can occur as a result of chronic or severe inflammation in atopic dermatitis and contact dermatitis, particularly owing to scratching. If the lesions are associated with changes such as epidermal atrophy, dermal sclerosis (induration) and loss of follicular ostia, scarring processes are to be considered, including burns, discoid lupus erythematosus ( Fig. 4 ), lichen sclerosus ( Fig. 5 ), scleroderma, and rarely, chronic graft-versus-host disease ( Fig. 6 ). Patients with systemic sclerosis can present with depigmented patches dotted with perifollicular pigmentation (classically described as “salt and pepper” pattern). This presentation can mimic or be confused with repigmenting vitiligo. Histologically, there is a loss of interfollicular but not follicular melanocytes. The key to distinguishing these dyschromic lesions from vitiligo is the presence of sclerosis (induration), which can be visualized histologically and palpated clinically.
Related posts:
The Medical Treatment of Vitiligo
Quality of Life, Burden of Disease, Co-morbidities, and Systemic Effects in Vitiligo Patients
Skin Cancer Risk (Nonmelanoma Skin Cancers/Melanoma) in Vitiligo Patients
Surgical Therapies for Vitiligo
Depigmentation Therapies for Vitiligo
Vitiligo Pathogenesis and Emerging Treatments
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