Vitiligo is an autoimmune disease in which pigment cells (melanocytes) disappear from the skin, resulting in slowly progressing patches of depigmented skin throughout the body.
Other autoimmune diseases occur in 10-15% of vitiligo patients. The most common are thyroid autoimmune diseases.
Treatment is most successful if it is started at an early stage of the disease when the melanocytes have not yet disappeared completely.
The treatment takes several months, and usually the re-pigmentation is not complete.
Vitiligo patches tend to sunburn easily.
Vitiligo impairs the psychosocial well-being of patients.
Epidemiology and pathophysiology
Vitiligo affects about 1% of the population. Vitiligo is encountered in all ethnic groups and equally between men and women.
Almost half of patients are under 20 years of age at disease onset.
According to different studies, 10-50% of close relatives of patients with vitiligo have vitiligo or other autoimmune diseases.
The mode of inheritance for vitiligo is not known. Multiple susceptibility loci have been identified in genes regulating immunology and melanocyte function and in the HLA gene region. Regulation disturbances affecting multiple genes are probable as is incomplete penetrance.
Autoimmune reactions and oxidative stress are probably the most important pathogenetic mechanisms in the development of vitiligo. Autoreactive cytotoxic CD8-positive T cells destroy epidermal melanocytes. The underlying precipitating factor is unknown.
Skin damage or sunburn may trigger vitiligo (Köbner phenomenon). Many patients connect the onset of vitiligo with pregnancy or mental stress.
Symptoms and findings
Vitiligo patches (pictures F1F2F3) usually occur symmetrically in the limbs (pictures F4F5F6).
The primary predilection sites on the trunk are the umbilical area and around the nipples. In the face depigmentation characteristically occurs around the mouth and eyes.
Light-coloured patches may also occur in the scalp hair or in hair on other areas of the body.
The patches usually progress gradually, and spontaneous repigmentation occurs only rarely.
In children, halo naevi (picture F7) may precede later development of vitiligo.
Segmental vitiligo, which is unilateral and linear or blotch-like, occurs more rarely. It develops quickly in about six months and then ceases to progress.
The depigmented areas are prone to sunburn and hence they must be protected with clothing or sunscreen with a SPF of at least 30.
Thyroid functionExamining a Patient with a Thyroid Complaint (thyroid abnormalities are present in about 20% of cases, but the symptom onset does not usually coincide with that of vitiligo)
TSH, free T4
TPO antibodies and antithyroglobulin antibodies. These identify patients who have an increased risk of developing autoimmune thyroiditis.
As required: basic blood count with platelets, antinuclear antibodies, vitamin B12 and fasting blood glucose, and other tests based on the patient's symptoms
Differential diagnosis
Diagnosis is made on the basis of clinical findings. If necessary, histopathological testing may be used to confirm diagnosis: melanocytes have disappeared from the epidermis.
The skin lesions in pityriasis versicolor Pityriasis Versicolor are pale in the summer and may be confused with vitiligo. Pityriasis versicolor lesions are associated with slight scaling which is not a feature in vitiligo.
Pityriasis alba is a patchy pigment deficiency, especially in children with atopic dermatitis. This is probably due to postinflammatory hypopigmentation.
Lichen sclerosus of the vulva or penis Lichen Sclerosus may resemble vitiligo. A biopsy will assist in diagnosis.
Idiopathic guttate hypomelanosis is an age-related, small-patch loss of pigmentation occurring in areas of skin exposed to UV radiation from the sun.
Treatment
No definitely effective treatment is available for vitiligoand after successful treatment, vitiligo recurs easily.
The aim of treatment may be to stop the expansion of vitiligo and/or to restore the pigment of the patches, or to prevent the recurrence of symptoms.
Signs of active and progressive vitiligo include spotty confetti-like pigment loss, residual but subdued pigment at the borders of the vitiligo patches and Koebner's phenomenon, i.e. pigment loss in the area of a skin lesion.
Treatment is most successful if it is started in an early stage of the symptoms, when the melanocytes have not yet completely disappeared from the epidermis.
In segmental vitiligo the treatment result is especially poor.
It may be decided, in agreement with the patient, not to attempt to treat vitiligo.
In light skinned patients, the patches are hardly noticeable during the winter months.
Many patients are content if the most visible lesions can be camouflaged with specially chosen make-up or self-tanning cosmetic products.
The patient must protect his/her skin against the sun as vitiligo patches are prone to sunburn, and if unaffected skin areas become suntanned the contrast will be emphasized.
The treatment response is based on immunosuppression and melanocyte regrowth and activation.
For small-area vitiligo on the trunk or limbs, potentglucocorticoid ointmentsare usedonce daily for a maximum of 3 months or in 2-week intervals, with a 2-week break in between, for a maximum of 6 months. Patients must be monitored for potential dermatological adverse effects of glucocorticoids.
For facial and thin skin areas, such as flexural surfaces, and in children, tacrolimus is the first-line topical treatment of vitiligo. The recommended period of treatment is at least 6 months, with dosing twice daily.
Maintenance treatment twice a week for several months with tacrolimus ointment or a strong glucocorticoid ointment is possible to prevent recurrence of symptoms if repigmentation has been achieved.
Narrow-band UVB phototherapy is used when vitiligo covers > 15-20% of the surface area of the skin and/or is expanding.UV therapy is usually combined with topical treatment using a glucocorticoid ointment.
A glucocorticoid can be used orally as a short course or as a pulse therapy to slow down rapidly progressing vitiligo.
The treatment response is best in young patients with vitiligo that has appeared recently. The response is usually weakest on limbs and best on the face.
New treatments, such as Janus kinase (JAK) inhibitors and surgical treatments (melanocyte transplantation) for localized vitiligo, are being developed.
Quality of life
People with vitiligo often experience stigmatization, social isolation and low self-esteem.
Vitiligo is associated with anxiety and depression.
Vitiligo impairs the patient's quality of life to the same degree as psoriasis, atopic dermatitis or hand dermatitis.
In some cultures vitiligo is considered particularly traumatic. A historical/cultural explanation can probably be found in the differential diagnosis: white patches are also associated with leprosy and late-stage syphilis.
A specialized care unit can be consulted regarding treatment options for vitiligo, if needed.
An open discussion with the patient and the parents of a paediatric patient about the limitations of treatments can help to reach shared decision-making and realistic goals.
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