Guidelines for GPs on the Diagnosis and Treatment of Vitiligo*
Vitiligo is a common disease that results in depigmented skin. It can develop at any age; the average age of onset is about 20 years. There is evidence to suggest that in some patients vitiligo is an autoimmune disease and that it shows a familial trait in about 18% of cases. Vitiligo occurs in all races and has an estimated prevalence of 0.5-1%. It can cause a great degree of psychological distress.
Some patients report spontaneous repigmentation, but this is rare. More typically, vitiligo is a chronic persistent disorder with a pattern of progress that features long periods when the disease is static and relatively inactive, interspersed with shorter periods when areas of pigment loss extend. There is a shortage of effective treatments and almost all those available have been borrowed from therapies whose prime target is another disease.
The initial approach to a patient who is thought to have vitiligo is to make a definite diagnosis, offer psychological support, and suggest supportive treatments such as the use of camouflage cosmetics and sunscreens. The options of having treatments (or no treatment) may then be considered.
To start with, the diagnosis of vitiligo has to be confirmed. Most commonly, vitiligo produces symmetrical depigmented areas of skin that otherwise appear normal. Vitiligo can affect melanocytes in the hair roots resulting in patches of white hair. Depigmentation can affect mucosal areas such as in the mouth or genitalia.
Diagnosis is more straightforward with the symmetrical type of vitiligo than with the less common segmental form in which unilateral depigmentation develops. In patients with an atypical presentation, diagnosis is more difficult and referral for expert assessment by a dermatologist is recommended.
Three main diseases can be mistaken for vitiligo:
- Tinea versicolor, a superficial yeast infection that can cause loss of pigment on the upper trunk and chest. Darker skinned individuals show hypopigmented patches with a fine dry surface scale.
- Piebaldism, an autosomal dominant disease in which there is absence of melanocytes from the affected areas of the skin, a forelock of white hair is usually evident and can be present at birth.
- Idiopathic guttate hypomelanosis, in which multiple small, white macules are found, mostly on the trunk or on sun exposed parts of the limbs.
The doctorâ€™s examination should include observation of the disease distribution, extent, whether depigmentation is total or partial, if there are symmetrical lesions, and if there is involvement of mucous membranes.
In adults with vitiligo, a blood test to check thyroid function should be considered in view of the high prevalence of autoimmune thyroid disease in patients with vitiligo.
The doctor should also record the effect of vitiligo on the patient's quality of life. In some people, especially those with a white skin colour, vitiligo may cause relatively little concern. However, psychological effects can be underestimated. Depigmentation on the face or hands is often visible to others, so vitiligo can be devastating and have a significant impact on the patientâ€™s quality of life and self esteem. It may cause social isolation and significant depression, create difficulties in sexual relationships, stigmatisation and affect perceived suitability for marriage.
Patients should be given information about the Vitiligo Society in the UK or other national patient help organisations.
Patients should be offered advice about sunscreens and cosmetic camouflage including fake tanning products.
Deciding on appropriate treatment
For adults and children with skin types I (always burn, never tan) and II (always burn, sometimes tan) it may be appropriate to consider, after discussion, whether the initial approach may be to use no active treatment other than sunscreens and camouflage cosmetics.
Treatment of vitiligo can be viewed in two phases: the first is to halt the progression of the disease; and the second is to induce repigmentation. Some treatments can be considered to achieve both.
Doctors should discuss treatment options, bearing in mind that they are generally unsatisfactory. Recent research indicates that the effectiveness of treatments depends not so much on a personâ€™s age but where the vitiligo is located and when it started. To this end, the mnemonic FACES provides a useful aide memoire for the best prognosis in treating vitiligo, ie treat the Face, when A Child, the Early manifestation of vitiligo, and Small areas.**
Treatments available to non-specialists:
The use of topical steroids is the usual first line treatment. Recommended treatments for children differ slightly from those for adults:
In adults with recent onset of vitiligo and in children, treatment with a potent (betamethasone valerate) or very potent (clobetasol propionate) topical steroid should be considered. These can repigment vitiligo, but only in a small proportion of cases. A trial period of no more than 2 months is recommended, as skin atrophy has been a common side effect.
Topical calcineurin inhibitors
Calcineurin inhibitors have an advantage over steroids in that they do not thin the skin, which is a concern to many patients. In adults with symmetrical types of vitiligo, topical pimecrolimus should be considered as an alternative to a topical steroid. The side effect profile of topical pimecrolimus is better than that of a highly potent topical steroid, although stinging may occur in some cases.
In children, topical pimecrolimus or tacrolimus should be considered as alternatives to a highly potent topical steroid in view of their better safety profiles.
An assessment needs to be made after 2 months to establish whether or not there has been a response to the above treatments.
Patients whose condition is difficult to diagnose, unresponsive to straightforward treatments, or is causing psychological distress, are usually referred to a dermatologist.
Phototherapy is appropriate for extensive vitiligo, especially if it is active. It has been a mainstay of treatment for several years. There is evidence that some vitiligo patients respond well to phototherapy with narrow band ultraviolet B (NB-UVB). Also research indicates that NB-UVB is more effective than oral PUVA (psoralen in combination with UVA). Phototherapy treatments should be considered only in specialist dermatology units.
Depigmentation (p- (benzyloxy) phenol (hydroquinone monobenzyl ether))
Where a patient has extensive vitiligo with a dark skin tone, especially on a cosmetically sensitive area such as the hands or face, it is worthwhile considering whether complete depigmentation of the affected areas might be beneficial. The profound effect that this may have culturally needs to be taken into account and fully understood by the patient. Depigmentation treatments should be reserved for severely affected adults and should be undertaken only by specialist dermatologists.
Surgical treatment of vitiligo is appropriate for cosmetically sensitive sites, for example, on the face or back of the hands, but only if the disease has been inactive for 6-12 months. Surgical treatments are not recommended in children and should be considered only in specialist dermatology or plastic surgery units.
Since vitiligo is viewed as an autoimmune disease, it is natural to wonder whether systemic treatment might have something to offer. However, at present, the use of oral dexamethasone to arrest the progression of vitiligo cannot be recommended due to an unacceptable risk of side effects.
Cognitive therapy and psychological support
Vitiligo is a disease which can have profound psychological effects, and clinicians should make an assessment of the psychological and quality of life effects of vitiligo on adults and children. Psychological interventions should be offered as a way of improving coping mechanisms in patients severely affected by vitiligo. Cognitive behavioural therapy strategies rather than avoidance or concealment may be associated with better coping. Parents of children with vitiligo should be offered psychological counselling.
* These guidelines are mainly a summary of the article Vitiligo: concise evidence based guidelines on diagnosis and management by David J. Gawkrodger, Antony D. Ormerod, Lindsay Shaw, et al, Postgrad Medical Journal 2010 86: 466-471.
** Information about this research, conducted in Japan, is taken from a poster presentation, Evaluation of our recent therapies of vitiligo vulgaris by Shibata, T. et al, at the 21st International Pigment Cell Conference, 20-24 Sept. 2011 Bordeaux, France.