Vitiligo

Vitiligo (white patches) is a fairly common pigmentation disorder, the main symptom of which is white patches on the skin. This is caused by the loss of melanocytes (cells that produce the skin pigment melanin) in the epidermis. Rashes can appear anywhere on the body and the spots can merge together to form large areas of depigmentation. The starkest contrast between healthy and damaged skin is observed in people with dark skin. People with vitiligo often experience anxiety, feel rejected by others and lack self-confidence.

Whiteness is the most common cause of depigmentation. It is estimated that up to 2% of the general population is affected, regardless of gender, ethnic origin or other epidemiological factors. Whiteness can occur at any age, but the most common age at which rashes appear is between 20 and 30 years. Nearly one third of patients are children, and about 80% of adults have their first skin lesions before the age of 30.

Causes of vitiligo

The causes of vitiligo are unknown. Patients often attribute the onset of the disease to emotional stress, illness, trauma, sunburn and pregnancy. However, there is no scientific evidence to support the influence of these factors on the development of vitiligo.

It has been observed that patients with vitiligo, as well as their first-degree relatives (parents, siblings), are more likely to have other autoimmune diseases. Therefore, it is thought that vitiligo may also be an autoimmune skin disorder. In addition, people who have undergone a bone marrow transplant have an almost 3-fold increased risk of developing whiteheads.

As already mentioned, in vitiligo, white patches on the skin are caused by the loss of melanocytes. There are several theories that attempt to explain the possible causes of melanocyte loss, the most popular of which are:

• Genetic predisposition
• Autoimmune processes
• Influence of oxidative stress
• Viral infection
• Biochemical reactions
• Influence of neurotransmitters
• Melanocyte pathology

Although research evidence supports the role of autoimmune reactions and oxidative stress in the development of vitiligo, none of these theories can explain the multifaceted manifestations of the disease. In contrast, the so-called “convergence” theory suggests that the destruction of melanocytes is caused by the interaction of several of the above factors.

• Genetic predisposition. 25- 50% of people with whiteheads have family members who also suffer from this skin disease. 6% of patients report that their siblings have vitiligo.
• Autoimmune diseases. The association between vitiligo and other autoimmune diseases such as autoimmune thyroiditis, Graves’ disease, type 1 diabetes, focal alopecia, rheumatoid arthritis and psoriasis has long been established. It has been estimated that as many as 20% of white people may have autoimmune thyroid disease. Autoantibodies are produced in the body against a specific target organ such as the thyroid gland. Such autoantibodies are also produced against melanocytes, resulting in their destruction. In addition, other changes in the body’s immune system play a role.
• Melanocyte pathology. One theory of vitiligo is that melanocytes may die as a result of toxic compounds produced during the synthesis of melanin (skin pigment). The cells’ susceptibility to these compounds is increased by oxidative stress. The theory is supported by long-term observations that contact with certain chemicals (e.g. industrial pollutants, harsh household chemicals, etc.) eventually leads to changes in skin pigmentation. Another reason for the death of melanocytes in white skin disease is the “detachment” of melanocytes from the basement membrane (the anatomical structure that separates the epidermis from the true skin). This can be caused by trauma, free radicals, autoimmune reactions, etc.
• Oxidative stress. Several scientific studies have shown that oxidative stress can be the start of a chain reaction leading to the death of melanocytes. The balance between oxidation and reduction reactions in the cell is disrupted, antioxidants are significantly reduced and free radicals are increased. In people with a genetic predisposition to vitiligo, oxidative stress can lead to the onset of the disease by influencing the immune response to external and internal factors affecting the body.
• Neurotransmitters. This theory suggests that nerve endings near the pigment cells may release substances that have a toxic effect on melanocytes. It has been observed that in some cases, vitiligo rashes are arranged according to dermatomes (i.e. the area of the skin that is innervated by a particular nerve). There are also cases of vitiligo following certain diseases of the nervous system, such as viral encephalitis, multiple sclerosis and peripheral nerve damage.

Vitiligo symptoms

General signs of vitiligo. The most characteristic sign of vitiligo is a white patch of skin. They do not cause any unpleasant sensations – they do not scratch, burn or hurt. They can vary in colour from milky white to chalky white. There are no signs of inflammation. The spots vary in size and may sometimes merge together to form large areas of depigmentation with distinct borders. Vitiligo can affect any part of the body, but the most common areas are the face, arms and torso. Often, patients attribute the onset of the disease to recent emotional stress, burns or other trauma to the skin, pregnancy. Vitiligo can affect both children and adults.

Vitiligo spots can have several shades: white, light brown, milky white, chalky white, etc. The colour variations are most pronounced in dark-skinned patients.

The hair in the affected area also loses pigment. In some cases, vitiligo can manifest itself in loss of pigment in the scalp, eyebrows and eyelashes. It has been observed that patients with vitiligo and their relatives have a faster fading of skin colour.

Halo moles indicate a higher risk of developing vitiligo. It has been reported that 6-26% of patients with halo moles develop vitiligo during their lifetime.

According to the age of onset, there are 2 types of vitiligo:

• The first type is characterised by early onset of the disease (usually before the age of 12 years), early onset of lividity, Halo moles, first-degree relatives with vitiligo, and the Kiobner phenomenon (see below).
• The second type is adolescents or young adults who often have other autoimmune diseases.

Classification of vitiligo

Vitiligo is divided into two broad categories: non-segmental and segmental.

Non-segmental vitiligo can be:

• Generalized. The spots are symmetrically distributed on both sides of the body. Different parts of the body are affected. This form of the disease may begin in childhood or early youth. Rashes appear on areas of the body that are frequently traumatised, rubbed or otherwise mechanically exposed. The face, torso and limbs are often affected. As the disease progresses, most of the pigment is lost and almost all of the skin is affected, with only patches of healthy skin remaining. This form is then called the universal form.
• Acrofacial, acral. The spots appear on the face and/or limbs. Later, the rash may spread over the whole body.
• Mucosal. This form of the disease affects the mucous membranes of the mouth and genitals. The rashes may occur against a background of generalised vitiligo or may occur alone.
• Hypochromic. More common in dark-skinned people. Only part of the pigment is lost, so the affected skin appears only slightly lighter than the surrounding healthy skin.

Segmental vitiligo. The rarest type of vitiligo. Characterised by a skin lesion at least partially corresponding to a specific dermatome. The area of depigmentation appears over several years and stabilises, the skin lesion does not tend to spread. In addition, there is early damage to the hair. Several skin segments of the body may be affected at the same time, not necessarily on the same side of the body.

Kiobner phenomenon. Frequent traumatisation of the skin, e.g. rubbing, scratching, as well as frequent contact with allergenic and irritating substances, can provoke the appearance of white patches, especially on the neck, elbows and ankles. It is worth noting that this phenomenon is not specific to vitiligo alone.

Interestingly, in 30-40% of cases, vitiligo can also affect melanocytes in the retina, inner ear and meninges. Fortunately, it does not cause any symptoms and is completely harmless.

Diseases associated with vitiligo

Autoimmune disorders. As already mentioned, vitiligo is often associated with autoimmune diseases such as, Autoimmune thyroiditis, focal alopecia, psoriasis, type 1 diabetes, rheumatoid arthritis, inflammatory bowel disease, scleroderma, myasthenia gravis, discoid and systemic lupus erythematosus, Sjögren’s syndrome, etc… It has been shown that people with the above diseases have a much higher likelihood of developing generalized vitiligo than the general population.

Genetic syndromes. Vitiligo is a component of extremely rare genetic syndromes such as Kabuki, Alezzandrini, Vogt- Koyanagi-Harada. These syndromes are characterised by lesions of organs other than the skin.

Psychosocial problems. Often, whiteness causes a lot of psychological stress, lowers self-confidence and leads to the development of various complexes. The negative impact on mental health and the emotional burden of the disease are more acute for women and darker-skinned people. Affected children often suffer from impaired social adaptation in their peer group, leading to the development of various psychological problems.

The course of the disease

The course of vitiligo is unpredictable. In some patients the rashes remain stable, while in others they progress over the course of life. In most cases, the location of the spots and the area of affected skin change over the years, with the spots getting larger and new skin lesions appearing. Disease progression is more common in these cases:

• When there is a family history of white skin disease
• High disease duration
• Kiobner phenomenon is detected
• Mucosal lesions are present

However, in most cases disease progression is unpredictable.

Vitiligo diagnosis

The diagnosis of vitiligo is usually not difficult. The disease is diagnosed on the basis of the characteristic features – congenital, clearly demarcated, uniform white patches with a distinct outline, separated from the surrounding healthy skin, and no signs of inflammation. In the diagnosis of vitiligo, the following are assessed:

• Age of onset
• Factors that may have provoked the onset of the disease
• Other symptoms associated with the rash
• Spread of rashes
• Changes in the rash over time
• Comorbidities
• Medications used
• Patient’s occupation, possible contact with chemicals
• Diseases of family members

Auxiliary diagnostic methods. Examination with a Wood’s lamp is often used to diagnose vitiligo. This is a special device that emits light of a certain wavelength. When the affected area is illuminated with it, the spots only become more pronounced, taking on a bright bluish-white hue. Other dermatological tests, such as dermatoscopy, skin biopsy and histological examination, are usually not necessary. Thyroid function tests are often recommended because of the relatively frequent association of the disease with autoimmune thyroid pathology.

Treatment of vitiligo

There is as yet no definitive cure. Current therapies only slow down the progression of the disease and may lead to the reappearance of pigmentation. It is important to assess the following before prescribing treatment:

• Age of onset
• The form of vitiligo (segmental, non-segmental)
• Mucosal status, Kiobner phenomenon
• Speed of disease progression, spread of spots
• Previous episodes of pigmentation recovery (repigmentation)
• Previous treatments and response to them
• Family history of vitiligo and other autoimmune diseases
• Any comorbidities
• Medications and food supplements taken
• Patient’s occupation, possible contact with chemicals
• Impact of the disease on the patient’s quality of life

It is very important to examine the skin of the whole body for possible lesions. Vitiligo “likes” to affect the lips, the skin around the mouth, the eyes, the dorsal surface of the hands, the fingers, wrists, elbows, armpits, nipples, navel, sacrum, groin, knees. The most appropriate and effective treatment method is determined by determining the area of the affected skin. Vitiligo has an unpredictable course and a slow response to treatment, which is a real challenge for both the doctor and the patient. Different areas of the body respond differently to treatment, e.g. spots on the face and torso regain pigmentation slightly faster than rashes on the arms. In addition, the treatment effect is most evident in darker skin tones.

The severity of the disease, the patient’s preferences (cost, availability) and the expected response to treatment also influence the choice of treatment method. Several treatments (e.g. phototherapy and oral or applied corticosteroids) have a better effect when combined than when used alone. Unfortunately, sometimes the disease recurs despite treatment and, in addition, no treatment method can guarantee that the recurrent pigmentation will persist permanently.

Main methods of vitiligo treatment

Topical corticosteroids. These are among the most common drugs used to treat vitiligo. They are suitable when the foci of the disease are few and small. A better treatment effect is achieved when these drugs are combined with phototherapy. However, prolonged use of corticosteroid ointments can lead to folliculitis, skin atrophy and telangiectasias. Topical corticosteroids are not recommended for facial vitiligo and when large areas of skin are affected.

Topical calcineurin inhibitors. These are immunomodulatory drugs that inhibit the production of various inflammatory molecules. In addition, they do not cause skin atrophy and are therefore safe to use on the face.

Phototherapy. This is treatment with sunlight or artificial ultraviolet rays from special medical devices. Narrow-wave UVB phototherapy is the most commonly recommended treatment for vitiligo, which stimulates the production of melanocyte-stimulating hormone, activates fibroblasts and other growth factors, and stimulates the proliferation of melanocytes and melanin production. Narrow-wave UVB phototherapy has no systemic toxic effects and is safe for children and adults. This method is used for both disease stabilisation and repigmentation. PUVA therapy, which has been widely used in the past for the treatment of vitiligo, is also widely used for the treatment of vitiligo as it stimulates the production of the pigment melanin and inhibits inflammatory processes in the skin.

Systemic corticosteroids. Low doses of oral corticosteroids can be administered to slow the progression of rapidly progressive vitiligo. Often this approach is combined with phototherapy. However, these drugs are not effective for repigmentation when vitiligo is stable.

Alternative treatments. Antioxidants and vitamins are often recommended for vitiligo patients as an additional means of improving skin condition. There are few scientific studies on the effect of dietary supplements on repigmentation, but it has been observed that vitamin C, vitamin B12 and folic acid can help to stabilise the course of the disease and to promote the recovery of skin pigmentation. Alpha-lipoic acid is another active substance, an antioxidant, which, in combination with vitamin E and polyunsaturated fatty acids, may also accelerate repigmentation. Ginkgo extract has been used since ancient times in Chinese folk medicine to treat various neurological disorders, vascular pathologies and skin diseases. Small sample studies have shown that the progression of vitiligo is significantly slowed down and pigmentation is restored in most areas of affected skin when ginkgo extract is used.

Cosmetic camouflage is suitable for patients with vitiligo on the face, neck and hands. It includes cosmetic products with tint, self-tanning products. Tattoos, permanent make-up are procedures to be avoided as they increase the risk of Kiobner’s phenomenon and tattoo pigment can provoke further changes in skin colour.

Prognosis of vitiligo

Vitiligo is a chronic disease, the course of which is quite difficult to predict. Vitiligo that occurs at an early age is associated with faster progression and more damage to the body surface. Most patients manage to maintain a stable disease course. In some cases, the pigment recovers spontaneously.

In all cases, it is recommended to consult a dermatologist who will assess your skin condition and prescribe the most appropriate treatment method so that you can enjoy healthy skin again.