Caroline Guignot
November 06, 2023
International vitiligo experts have published new recommendations in two parts — one dedicated to management algorithms, the other to specific treatment recommendations — to update the text from 2013. These recommendations are aimed at dermatologists and primary care providers and propose relatively simple algorithms. Julien Seneschal, MD, PhD, professor of dermatology at the University of Bordeaux in Nouvelle-Aquitaine, France, offers some clarification.
Univadis: These new recommendations recognize that diagnosing vitiligo can be difficult. Why is this so?
Seneschal: Vitiligo is a clinical diagnosis. Seasoned dermatologists can make this diagnosis fairly easily using a UV or Wood’s lamp. This lamp lets them see if melanocytes have been destroyed within presenting skin patches. The main differential diagnosis is pityriasis versicolor, where there is simply hypopigmentation, rather than no pigmentation. But in clinical practice, diagnosis can sometimes be delayed by several years, and it’s not rare to see people treated with antifungals on a long-term basis after being mistakenly diagnosed with pityriasis, especially in primary care settings. So, the first message is to reconsider diagnoses in treatment-compliant patients who see no response 4 to 6 months after starting medication.
The Wood’s lamp test also allows clinicians to look for signs of disease activity. In 20% of patients with a developing form of the condition, the borders of lesions are not clear; they are often blurred or have a confetti-like appearance. On this last point, the presence of depigmentation can aid clinicians in distinguishing vitiligo from the hypopigmentation seen in idiopathic guttate hypomelanosis, which is nonprogressive.
Univadis: You have also revised the definition of vitiligo.
Seneschal: Yes, to separate the algorithms for treating nonsegmental forms, which are combined under the generic term “vitiligo,” from segmental forms that must be defined as such. We should note that the former represent 90% to 95% of cases. The lesions are bilateral, symmetrical macules and occur in adults over the age of 30 years. Segmental vitiligo is rarer, occurring most commonly in children and teenagers, and refers to lesions in a single unilateral area of the body that appear in one single outbreak. We must remember that there are mixed forms where both types co-occur, firstly segmental vitiligo, then the nonsegmental form, and so-called universal forms, where more than 90% of the surface area of the body is affected. As a whole, these forms of vitiligo affect 0.5% to 2% of the population.
Univadis: What messages should be sent to patients after their diagnosis?
Seneschal: First, we need to challenge some misconceptions. There are three main ones. The first is that the disease is linked to stress. Although stress can aggravate the condition in predisposed individuals, it is not a triggering factor as such, since in 90% of cases, patients have an associated genetic predisposition to immune system mediators. Stress is a trigger in the same way that environmental factors are. Chafing is also a risk factor, which explains why vitiligo lesions are often located on the elbows, knees, or the underside of legs and arms.
The second misconception is that lesions must be protected from the sun. On the contrary, reasonable sun exposure is beneficial in promoting repigmentation, and depigmentation doesn’t increase the risk of skin cancer.
The last misconception relates to treatment: 65% of patients report having been told by their doctor that there is no effective treatment for their condition, which is wrong, as you can see in our recommendations. Additionally, although it’s thought that recent-onset vitiligo patches have a greater chance of becoming stable and returning to their usual pigmentation, treatment can be offered for older lesions.
Univadis: What should treatment strategies focus on?
Seneschal: Broadly speaking, the first objective is to stabilize the lesions, then try to repigment the stable lesions, and finally maintain this repigmentation. We propose two algorithms, one for nonsegmental vitiligo, the other for segmental forms. In all cases, the decision is taken as part of a shared medical decision: we need to align the patient’s aims — stabilization, repigmentation — with the options available.
The presence of white hair within the lesions themselves is, for example, a criterion of a poor prognosis or poor response to treatment that should be taken into account. It means that the follicular melanocyte stem cells are affected. When presented with a very generalized form, clinicians should still proceed gradually: determining for example an initial aim — an area of the body to be treated first — then moving forward step by step, depending on clinical improvement. It is thought that hydrating the skin can sometimes limit outbreaks, because doing so restores the skin barrier, although this has not actually been proven.
Beyond medical treatment, clinicians should not hesitate to offer their patients supportive care, in particular, psychological support for those who are anxious or stressed by such a visible condition. In terms of food supplements, taking an antioxidant such as gastroprotective superoxide dismutase seems to promote the repigmentation achieved using phototherapy in certain patients, but this supplement is not subsidized by social security in France.
Univadis: What are the main features of vitiligo treatment?
Seneschal: Most of the treatments used today to stabilize and repigment nonsegmental lesions are used off label. The first of these, which can be considered in a primary care setting after a diagnosis has been confirmed, is topical corticosteroids for lesions not located on the face. Once-daily application is advised in the evening five times per week, or sometimes 15 days per month. The efficacy can only be assessed after a minimum of 6 months of use. These drugs afford more than 50% improvement in 50% of patients, excluding lesions on the hands and feet, which are often difficult-to-treat areas.
For lesions on the face, we use tacrolimus, a topical calcineurin inhibitor and restricted drug that requiring a prescription from a dermatologist. Pigment is more easily restored to the face, and near-complete pigmentation is achieved in 80% of cases. Phototherapy is recommended as an adjunct to promote repigmentation. During the summer months, exposure to the sun without sun protection is advised for 15 to 20 minutes twice a week. During the winter months, phototherapy can be carried out in cabins or with an excimer laser for very localized lesions. When the disease is very active, general corticosteroid strategies may be adopted with mini-pulse therapy dosed according to patient weight administered on Saturday and Sunday mornings for 12 to 24 weeks. This strategy leads to stabilization of the disease in 80% of cases.
Other oral immunomodulators are used off label, such as methotrexate or cyclosporin, but evidence surrounding their efficacy is weak. Once pigmentation has been restored, maintenance treatment is needed, as 50% of patients will relapse to the same point within a year following treatment cessation. Twice-weekly application of tacrolimus to the face or of topical corticosteroids to the rest of the body is recommended. This helps to maintain restored pigmentation in 80% of cases. Phototherapy is less beneficial as maintenance therapy.
Univadis: The recommendations also introduce emerging therapeutic innovations, such as ruxolitinib, which received European approval in April 2023. What place will this JAK1 and JAK2 inhibitor have once it has been rolled out to French pharmacies?
Seneschal: Topical ruxolitinib alone has been compared with a placebo in two large phase 3 studies in more than 600 patients. Applied twice daily for 1 year, it affords more than 75% improvement in pigmentation when applied to the face in 50% of patients and more than 50% improvement in 50% of patients when applied elsewhere on the body. It is now being studied in combination with phototherapy. Ruxolitinib received market authorization in Europe in 2023 but has yet to be marketed. It paves the way for a real wave of innovation with other JAK inhibitors, such as upadacitinib, ritlecitinib, and baricitinib, which are currently being investigated for use in treating vitiligo. We are finally going to put an end to the idea that nothing can be done to treat vitiligo.
Key takeaways for primary care practitioners include the following points:
- Pay attention to differential diagnoses.
- Promote reasonable sun exposure without sun protection.
- Refer patients to a dermatologist if topical corticosteroids have been tried and failed.
Key takeaways for dermatologists include the following points:
- Treat active forms of the disease promptly.
- Treatment of children and pregnant women requires certain precautions, which are set out in the recommendations.
- A new generation of targeted treatments is arriving.
This article was translated from Univadis France, which is part of the Medscape professional network.
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