Vitiligo is a pigmentary disorder of the skin characterized by depigmented macules and patches. Vitiligo is a progressive disorder in which some or all of the melanocytes in the affected skin are selectively destroyed.
This is caused by stoppage of melanin formation by the melanocytes that result in depigmented macule without any other change in the skin. The macules vary in size and shape and can be located on any part of the body including the mucous membranes of the lips and genitals.
Vitiligo is relatively common, with a rate of 1-2%.
The onset is most commonly observed in persons aged 10-30 years.
Pathophysiology of Vitiligo
Vitiligo is thought to be caused by multifactorial genetic and nongenetic factors. The exact cause remains unknown though.
Multiple theories have been proposed which include
- Autoimmune and cytotoxic cause resulting in melanocyte dysfunction or destruction.
- Neurochemical mediator destroys melanocytes or inhibits melanin production.
- Oxidant-antioxidant mechanism – An intermediate or metabolic product of melanin synthesis causes melanocyte destruction.
- Intrinsic defect of melanocytes
A composite theory of all the factors together has been proposed.
Clinical Classifications of Vitiligo
Vitiligo may be divided into two groups: segmental and nonsegmental.
This type manifests as one or more macules that may follow the lines of Blaschko [Lines of normal cell development in the skin. These lines are invisible under normal conditions. They become apparent when some diseases of the skin or mucosa manifest themselves according to these pattern]
It is unilateral and does not cross the midline. Segmental vitiligo usually has an early onsetand is not associated with thyroid or other autoimmune disorders..
These types of vitiligo do not follow a segmental pattern.
This is characterized by one or more macules in a limited area
More widespread than focal vitiligo. Subtypes of are
Depigmentation occurs on the distal fingers and periorificial areas.
Scattered patches that are widely distributed.
Complete or nearly complete depigmentation of the body.
Trichrome / Quadrichrome
These are clinical variants of vitiligo.
Trichrome vitiligo is a clinical variant characterized by an intermediate zone of hypopigmentation located between the depigmented center and the peripheral unaffected skin.
Quadrichrome vitiligo has the presence of a fourth color (dark brown) at sites of perifollicular repigmentation
Patients have patches of skin color loss. While vitiligo may be more obvious in patients with darker skin, this disorder does not have a racial or ethnic predilection.
These patches are completely asymptomatic unless modified by treatment. Some of the lesions or some parts of the lesions may be hypopigmented and not completely depigmented and in some lesions the hairs may also be white (leucotrichia). The margins of the lesions are generally well defined and may be hyperpigmented in some cases.
Some patients have only a few small lesions which are static or progressing very slowly, while others have multiple lesions located all over the body. In some patients, the disease progresses very fast to cover the entire skin surface within a few months.
Thus, the course of the disease is very variable and in some patients even spontaneous repigmentation of the lesions may also be observed. The lesions are not usually present at birth, but may appear at any time thereafter.
The macules are chalk or milk-white in color and are well demarcated. Lesions can be round, oval, or linear in shape.
Lesions enlarge centrifugally over time at an unpredictable rate.
Face, neck, forearms, feet, dorsal hand, fingers, and scalp are most common areas affected.
Additionally, lesions may occur in regions frequently subjected to trauma, such as bony prominences, elbows, and knees. [Koebner phenomenon is the development of vitiligo in sites of trauma, such as a cut, burn, or abrasion. Koebnerization may occur in as many as 20-60% of vitiligo patients.]
- Alezzandrini Syndrome
- Chemical leukoderma
- Cutaneous Melanoma
- Dermatologic Aspects of
- Addison Disease
- Onchocerciasis (River Blindness)
- Tuberous Sclerosis
- Waardenburg Syndrome
- Halo Nevus
- Idiopathic Guttate Hypomelanosis
- Mycosis fungoides mimicking vitiligo
- Nevus Anemicus
- Pityriasis Alba
- Postinflammatory depigmentation
- Prior treatment with corticosteroids
- Tinea Versicolor
- Vogt-Koyanagi-Harada Disease
The diagnosis of vitiligo generally is made on the basis of clinical findings.
Biopsy may be helpful for differentiating vitiligo from other disorders of hypopigmentation.
Laboratory work for vitiligo may include the following:
- Thyroid panel
- Antinuclear antibody
- Antithyroid peroxidase antibody
Microscopic examination of involved skin shows a complete absence of melanocytes in association with a total loss of epidermal pigmentation.
Treatment of Vitiligo
In patients with lighter skin, no intervention may be needed. Instead, diligent sun protection may be the best strategy in order to avoid the surrounding normal skin from becoming more tan and making the lesions more obvious.
When therapy is necessary, topical steroids, topical calcineurin inhibitors, and narrow-band ultraviolet (UV)–B phototherapy are the mainstays of treatment. Treatment needs to be individualized.
There is no single effective therapy in all patients, and the response to therapy is highly variable.
Segmental vitiligo and onset < 14 years has been found to be associated with more refractory disease.
During therapy, pigment cells arise and proliferate from the pilosebaceous unit, and migrate up to 2-4 mm from the edge.
Phototherapy induces satisfactory repigmentation in a majority of patients with early or localized disease. A treatment period of at least 6 months may be necessary to accurately assess the responsiveness to the phototherapy
Narrowband UV-B (NB-UVB) has become the first choice of phototherapy for adults and children with generalized vitiligo.
Psoralen photochemotherapy, once mainstay has been replaced by Narrow band ultra violet B.
Psoralens combined with UV-A radiation is known as PUVA. Psoralens can be applied either topically or taken orally, followed by exposure to artificial UV-A radiation or natural sunlight. Adverse effects include phototoxic effects, nausea, and risk of skin cancer.
The excimer laser is an efficacious, safe, and well-tolerated treatment.
- Calcineurin inhibitors – Topical tacrolimus ointment
- Vitamin D analogs
- Calcipotriol, tacalcitol
- Target get the local immune response
- Influence melanocyte maturation and differentiation
- Synthetic analog of alpha-melanocyte–stimulating hormone
- Afamelanotide is delivered as a subcutaneous implant.
- Janus Kinase inhibitor therapy
- ruxolitinib 1.5%
- In cases with widespread involvement and failure of repigmentation
- 20% cream of monobenzylether of hydroquinone for 3-12 months.
Surgery may be indicated in segmental, focal or vitiligo in areas that tend not to repigment well like dorsal fingers, ankles, forehead, hairline.
For surgery a lesion must be stable [not actively progressing].
Following factors are indicators of stability
- No progression or growth for 2 years
- Spontaneous repigmentation
- A positive minigrafting test
- Repigmentation at 4-5 minigrafts
- The most accurate evidence
- Absence of new koebnerization [development of vitiligo in sites of trauma]
Noncultured epidermal suspensions
- Removal of the achromic epidermis
- Spread of previously prepared epidermal suspension onto the denuded area
- Cover with nonadherent dressings.
- Color mismatches common
Thin dermoepidermal grafts
- Removal of the depigmented epidermis by superficial dermabrasion
- Thin dermoepidermal sheets harvested with a dermatome are grafted
Suction epidermal grafting
- Epidermal grafts obtained by vacuum suction
- The recipient site can be prepared by suction, freezing, or dermabrasion of the sites 24 hours before grafting.
- The depigmented blister roof discarded
- Epidermal donor graft is placed on the vitiliginous areas.
- Small donor grafts are inserted into the incision of recipient sites
- Held in place by a pressure dressing.
Cultured melanocyte suspensions
- Depigmented skin is removed using liquid nitrogen, superficial dermabrasion, thermosurgery, or carbon dioxide lasers
- Very thin sheets of cultured epidermis are grafted or suspensions are spread onto the denuded surface.
- Tattooing can be used to repigment depigmented
- Color match is often poor
- Ezzedine K, Silverberg N. A Practical Approach to the Diagnosis and Treatment of Vitiligo in Children. Pediatrics. 2016 Jul. 138 (1):
- Ongenae K, Van Geel N, Naeyaert JM. Evidence for an autoimmune pathogenesis of vitiligo. Pigment Cell Res. 2003 Apr. 16(2):90-100.
- Oiso N, Suzuki T, Fukai K, Katayama I, Kawada A. Nonsegmental vitiligo and autoimmune mechanism. Dermatol Res Pract. 2011. 2011:518090.
- Toussaint S, Kamino H. Noninfectous papular and squamous diseases. Elder D, Elenitas R, Jaworsky D, Johnson B Jr. Lever’s Histopathology of the Skin. Philadelphia, Pa: Lippincot-Raven; 1997. 154-5.
- Bae JM, Jung HM, Hong BY, Lee JH, Choi WJ, Lee JH, et al. Phototherapy for Vitiligo: A Systematic Review and Meta-analysis. JAMA Dermatol. 2017 Mar 29.
- Grimes PE, Hamzavi I, Lebwohl M, Ortonne JP, Lim HW. The efficacy of afamelanotide and narrowband UV-B phototherapy for repigmentation of vitiligo. JAMA Dermatol. 2013 Jan. 149 (1):68-73.
- Rothstein B, Joshipura D, Saraiya A, Abdat R, Ashkar H, Turkowski Y, et al. Treatment of vitiligo with the topical Janus kinase inhibitor ruxolitinib. J Am Acad Dermatol. 2017 Jun. 76 (6):1054-1060.e1.
- Silverberg JI, Silverberg NB. Quality of life impairment in children and adolescents with vitiligo. Pediatr Dermatol. 2014 May-Jun. 31 (3):309-18.