Dr Shehnawaz Mir

Vitiligo is one of the oldest and commonest skin disorders affecting approximately 1-2% of the human population.The disease is characterized by the selective loss of melanocytes which results in typical nonscaly, chalky-white macules.Males and females are equally affected, although women and girls often seek consultation more frequently, possibly due to the greater negative social impact than for men and boys.

Vitiligo is a multifactorial disorder characterized by the loss of functional melanocytes. The “convergence theory” or “integrated theory” suggests that multiple mechanisms may work jointly in vitiligo to contribute to the destruction of melanocytes, ultimately leading to the same clinical result. These include genetic, autoimmune responses, oxidative stress, generation of inflammatory mediators and melanocyte detachment mechanisms.

Genetics of Vitiligo

• Around 20% of vitiligo patients have at least 1 first-degree relative with vitiligo, and the relative risk of vitiligo for first-degree relatives is increased by 7- to 10-fold.

• Monozygotic twins have a 23% concordance rate, which highlights the importance of additional stochastic or environmental factors in the development of vitiligo.

• Several corresponding relevant genes have now been identified which can increase the risk of developing vitiligo especially the ones involved in melanogenesis and immune regulation.

Types of Vitiligo

1. Generalized Vitiligo: It is characterized by white colored patches occurring in a random distribution over the entire body surface. It often affects areas that tend to experience pressure, friction and/or trauma eg elbows, knees, belt-line etc. It may begin in childhood or early adulthood.

2. Acrofacial vitiligo: It is characterized by white colored patches limited to the hand and feet and/or the face. It may later progress to include other body sites. The ‘lip-tip vitiligo’ is a sub-category of the acrofacial type in which lesions are restricted to the cutaneous lips and distal tips of the digits

3. Mucosal vitiligo: It typically involves the oral and/or genital mucosae.

4. Vitiligo universalis: It refers to complete or nearly complete depigmentation of the skin (80–90% of body surface). It is usually preceded by generalized vitiligo that gradually progresses to involve the entire body.

5. Focal vitiligo: It refers to a small, isolated, white patch without an obvious distribution pattern and which has not evolved after a period of 1–2 years.

6. Hypochromic vitiligo or vitiligo minor is characterized by the presence of hypopigmented macules in a seborrheic distribution on the face and neck associated with hypopigmented macules of the trunk and scalp.

7. Follicular vitiligo : It presents with leukotrichia in the absence of depigmentation of the surrounding epidermis.

8. Segemental Vitiligo (SV) : It refers to chalky white, non-scaly patch with distinct margins distributed in a segmental pattern. It has a rapid onset and is often associated with overlying white hair. The most commonly involved site in segemental vitiligo is ‘head’ typically involving the skin along the distribution of the ‘trigeminal nerve’.

Association of Vitiligo with other diseases: Many studies have demonstrated the associations of vitiligo with thyroid disorders and other associated autoimmune diseases, such as alopecia areata, rheumatoid arthritis, adult-onset diabetes mellitus, Addison’s disease, pernicious anemia, systemic lupus erythematosus, psoriasis and atopic background.

Diagnosis

The diagnosis of vitiligo is generally straightforward, made clinically based upon the finding of acquired, amelanotic, nonscaly, chalky-white macules with distinct margins.

The diagnosis of vitiligo does not usually require confirmatory laboratory tests. A skin biopsy or other tests are not necessary except to exclude other disorders that can mimic vitiligo.

Dermoscopy and Wood’s Lamp can sometimes be helpful to differentiate vitiligo from other depigmenting disorders.

Management of Vitiligo

The treatment of vitiligo is still one of the most difficult dermatological challenges. An important step in the management of vitiligo is to first acknowledge that it is not merely a cosmetic disease and that there are safe and effective treatments available. These treatments include phototherapy, topical and systemic immunosuppressants, and surgical techniques, which together may help in halting the disease, stabilizing depigmented lesions and stimulating repigmentation.

Choice of treatment depends on several factors including: the subtype of the disease, the extent, distribution and activity of disease as well as the patient’s age, phototype, effect on quality of life and motivation for treatment. The face, neck, trunk and mid-extremities respond best to therapy, while the lips and distal extremities are more resistant . Repigmentation appears initially in a perifollicular pattern or at the periphery of the lesions. Treatment for at least 2–3 months is needed to determine efficacy of treatment. UV light-based therapy is the most common treatment for vitiligo and, when combined with an additional therapy, is associated with an improved outcome.

Advice on cosmetic camouflage by a cosmetician or a specialized nurse should be offered and can be beneficial for patients with vitiligo affecting exposed areas. These include foundation-based cosmetics and self-tanning products containing dihydroxyacetone which provides lasting color for up to several days.

1. Topical Treatments : Topical Corticosteroids, Calcineurin Inhibitors (Tacrolimus, Pimecrolimus) are considered to be the first line treatment for localised vitiligo. Topical Vitamin D analogues like Calcipotriol can also be effective.

2. Oral Treatments : Oral Steroids in mini-pulse formulation can be used for arresting the progression of vitiligo.

3. Phototherapy : Due to its good safety profile in both children and adults and lack of systemic toxicity, NB-UVB has emerged as the initial treatment of choice for patients with vitiligo involving >10% of the body surface area.

Targeted phototherapy using 308-nm monochromatic excimer lamps or lasers is useful for the treatment of localized vitiligo. These devices deliver high-intensity light only to the affected areas while avoiding exposure of the healthy skin and lowering the cumulative UVB dose.

4. Surgical Treatment: Surgical methods can be offered as a therapeutic option to patients with SV and those with NSV with stable disease after at least a year of documented nonresponse to medical interventions and absence of Koebner’s phenomenon. The surgical techniques include tissue grafts (full-thickness punch, split-thickness and suction blister grafts) and cellular grafts (autologous melanocyte cultures and noncultured epidermal cellular grafts). Other techniques include cultured epidermal suspensions and hair follicle transplantation

What is new in the treatment of Vitiligo?

1. Topical Therapies like Afamelanotide, Bimatoprost lotion, tofacitinib gel, methotrexate gel

2. Topical ruxolitinib 1.5% cream

3. Oral Tofacitinib, Oral Apremilast

4. Fractional Co2 laser in combination with 5-Flourouracil cream

The psychosocial effect of vitiligo is important and well recognized .The skin plays an important role in our interaction with the world, and visible skin disorders can limit healthy psychosocial development owing to the stigma these disorders create. Historically, there has been a stigma attached to diseases of the skin and the people they affect. This psychosocial stress and these psychiatric comorbidities should be taken into consideration in vitiligo management, as stress can be a precipitating factor. Indeed, treatment of vitiligo should not be limited to the clinical disease severity but should also address the patient’s quality of life.

Vitiligo is a multivariate skin condition with a complicated pathophysiology. Despite recent significant advances in human knowledge of this condition, the origin and pathophysiology of vitiligo remain unknown. There are still questions about what causes melanocyte degeneration, and more research is needed to fully understand the etiology of vitiligo. Lastly, we as dermatologists, must keep ourselves updated with the latest innovations and treatment modalities to offer safe, reliable and positive experience to our patients with vitiligo.