Vitiligo
Vitiligo is a skin disease of unknown exact cause in which white areas appear on the skin as a result of pigment loss. Any part of the body can be affected, most commonly the face, lips, hands, arms, legs and genital areas. It occurs in 1% of the world’s population and affects men, women and all races equally.
Who Gets Vitiligo?
Vitiligo affects 1 or 2 in every 100 people; although it can appear at any age from infancy to old age, it begins before the age of 20 in half of patients, and 20% of patients have a family history.
Is vitiligo a disease of the internal organs? Is it contagious?
Vitiligo is not caused by any internal organ disease; for this reason, the general health of most patients is good. Vitiligo is not contagious.
What are the causes of vitiligo?
A pigment called melanin determines the colour of the skin, hair and eyes and is produced in cells called melanocytes. If these cells die or cannot produce melanin, the skin becomes lighter in colour or completely white.
Why this happens is not known, but there are 4 main theories:
1- There may be a genetic abnormality that causes damage to melanocytes
2- The body’s immune system may destroy melanocytes, perceiving the pigment as a foreign body and breaking it down.
3- Nerve cells that function abnormally may produce toxic substances that can damage melanocytes
4- Pigment-producing cells may destroy themselves. Toxic products may be produced while pigment is being made and may destroy the melanocytes.
Is vitiligo a treatable disease?
Vitiligo is a treatable disease, but because its exact cause is not known, treatment results may vary from person to person. Different treatment options are available, and these treatments
should be used for at least 3 months. The choice of treatment must always be decided together with a dermatologist.
Do herbal products have a place in the treatment of vitiligo?
Do not use any treatment (tablets or creams) that is claimed to be herbal but is not approved by the Ministry of Health; always consult your doctor.
Idiopathic Guttate Hypomelanosis
Idiopathic guttate hypomelanosis (IGH) is also known as “hypomelanosis of Cummins and Cottel”.
It results from insufficient melanin synthesis in melanocytes of the skin and hair follicles without a reduction in their number, a reduced melanin content and reduced transfer of melanin to skin cells. These are hypopigmented, porcelain-white, oval, polygonal, sharply demarcated spots 2–5 mm in diameter, developing in connection with a reduction in melanocyte number and usually located symmetrically on the outer surface of the forearms or the front of the legs. Their size does not change over time. Very rarely they may also be seen on the face. Their shape is limited by the existing skin lines. Very rarely the pigmentation may spontaneously return to normal.
It is common. It is seen in 46–70% of the population, in all races and skin types. It is also seen to affect darker-skinned people more often. It is acquired and very often confused with other skin diseases. The condition has been shown to increase in parallel with ageing. Clinically, more than 80% of cases are seen in people over 70.
Apart from its appearance, it causes the patient no discomfort. Although its frequency is the same in men and women, women more often seek an examination. It is easily diagnosed with a Wood’s lamp. Its cause is not fully known. However, because it appears in older age and is frequently located on the areas of the arms and legs that receive the most sun, chronic sun damage is thought to play a role. Cases of guttate hypomelanosis developing after PUVA/narrowband UVB have been reported.
Treatment
First of all, the skin should be protected from the sun. Light, superficial cryotherapy, microdermabrasion, treatments using micro skin grafts taken from normal skin, retinoids and fractional CO2 lasers can be used. Clinically successful results are obtained with excimer laser and Excilite 308 nm.
Ash Leaf Spots
Hypomelanotic macules are also characteristic, though not pathognomonic, skin lesions; they are also known as "ash leaf" spots. While other skin lesions may not appear until the 2nd to 6th decade, hypomelanotic macules are usually present at birth. They appear at some point in life in 90% of patients. They are seen better with a Wood’s lamp. Unlike vitiligo, the number of melanocytes and keratinocytes is normal, while melanin is reduced or absent.
Pityriasis Alba
Pityriasis alba (PA) consists of hypopigmented patches usually seen on the face and neck of children. In children, 97% of cases occur under the age of 3. On the face it is most often seen on the forehead and cheeks, and less often around the corners of the mouth and the eyes. The lesions are asymptomatic, meaning they cause no discomfort apart from their appearance. Very rarely, particularly in children, mild itching may be reported. The general health of these children is very good, and there is no systemic disease. PA usually appears as 2–3 lesions ranging from 0.5 to 5 cm in diameter. At first they are light pink, with slightly raised borders.
The pinkness then fades, leaving a hypopigmented lesion with slight fine scaling. Although the hypopigmentation disappears spontaneously over time, hypopigmented lesions can persist for a long time. Particularly in people with atopy they may last for 6 months, and cases lasting even 6 years have been reported.
The lesions favour periods of low humidity and become more noticeable in summer. In particular, tanning of the surrounding normal skin makes them more visible. PA usually recurs in the same places. Although it occurs in all skin types, it is usually more noticeable in darker skin. PA affects men and women equally and is common worldwide without geographical distinction. However, an association with schoolchildren and low socio-economic status has been shown. A hyperpigmented clinical variant of PA is seen, with a purplish plaque surrounded by a hypopigmented ring. Over time this parallels the development of fungal infections on the lesion.
Although many causes have been suggested for the development of PA, dry skin and atopy are the most convincing explanations. Aggravating environmental conditions include sun, low ambient humidity, wind, hot water and long baths, not using sunscreen, and soap use. It has been suggested that low serum copper levels may play a role in the development of PA (copper plays a role in melanogenesis). In PA lesions, melanocytes and melanogenesis are reduced. Contrary to earlier belief, it has been understood that there is no problem with the transfer of melanosomes to keratinocytes.
Treatment
- In mild cases, treatment is in fact not needed, because the condition is self-limiting and disappears on its own.
- Aggravating factors should be reduced, for example by avoiding the sun and using sunscreen, not using soap and limiting hot baths.
- The effective treatment is the use of moisturisers.
- During the erythematous phase, low-potency corticosteroid creams can be used.
- Topical forms of retinoids can be used.
- Because pigmented PA is associated with fungal infections, topical antifungals should also be added to treatment.
Pityriasis Versicolor
Pityriasis versicolor is a long-lasting, frequently recurring superficial fungal infection, seen all over the world, that settles in the outermost layer of the skin, the stratum corneum. It is also called tinea versicolor, but the term pityriasis versicolor is considered more appropriate. In Turkish it is popularly known as “samyeli”. Pityriasis means finely scaling, and versicolor means having a variable colour.
The yeast group that causes the infection, “Malassezia furfur”, is in fact part of the normal microorganism flora of the skin. “Pityrosporum orbiculare and Pityrosporum ovale” are actually two different morphological forms in the life cycle of Malassezia furfur. Malassezia furfur absolutely requires fatty acids to grow, so the disease favours areas with more sebum, the body’s oily secretion. Under suitable conditions this saprophytic yeast can change to a parasitic form associated with clinical disease. Factors causing this change include heat, a humid environment, hereditary predisposition, suppressed immunity and poor nutrition. For this reason pityriasis versicolor is also regarded as an opportunistic infection.
There are different views on how contagious the disease is. According to some researchers, the disease can be transmitted through close contact and shared items. On the other hand, others argue that Malassezia furfur is found in the normal flora and that a genetic predisposition and favourable environmental conditions are needed for infection to develop. Observations have shown that person-to-person transmission does occur. Transmission is also possible directly or through contaminated clothes and bed sheets. Transmission between spouses has been observed very rarely. Experiments on transmitting the disease to healthy people by direct inoculation have shown that this is not possible.
Clinically, there are no clear data on exactly how and under what conditions Malassezia furfur, which lives as a yeast in the normal skin flora without causing disease, changes into its parasitic, disease-causing form. However, the factors that prepare the ground for this change have been grouped under two headings: factors related to the patient and factors related to the environment. Patient-related factors include genetic predisposition, oily skin, excessive sweating, removal of the adrenal gland (adrenalectomy), Cushing’s disease, diabetes mellitus, pregnancy, malnutrition, severe burns, steroid treatment, immunodeficiency and contraceptive pills. Environmental factors include excessive heat (seasonal and regional) and increased humidity.
In addition to these factors, the disease has been found to develop most often in areas covered by clothing, suggesting that Malassezia furfur may more easily acquire the capacity to cause disease because of the higher amount of carbon dioxide in covered areas.
Anatomically, the clinical picture develops in areas with sebaceous glands: the chest, shoulders, back, upper part of the upper limbs, neck and abdomen. Although rare, the infection may also develop on the face, scalp, genital area and lower limbs. In children, the face is known to be the most commonly affected area. The infection is never seen on the palms and soles, where there are no sebaceous glands.
It is common especially in the summer months and in tropical regions. For example, in the Rio Grande do Sul region of Brazil, 36% of the population is affected by P. versicolor. In Western Samoa this rate rises to 50%. Quite high rates are also seen in the coastal regions of Mexico. In temperate climates its rate among all dermatological diseases is around 0.5% to 4%. People who sweat a lot and have thin skin are reported to be at risk. Its occurrence mostly in the summer months highlights the importance of sweating and increased ambient humidity. A familial predisposition to pityriasis versicolor is known. It has been noticed that familial predisposition is generally found in patients with oily skin and excessive sweating. It is reported that human skin begins to be colonised by Malassezia furfur at the end of childhood and the start of puberty, in parallel with the activity of the sebaceous glands.
P. versicolor is mostly seen in adults and less often in children and the elderly. It is reported to be seen more often in newborns than in children. Late adolescents and young adults are affected much more frequently. Men and women are affected equally. In young children and the elderly, infection occurs in special circumstances such as suppressed immunity.
The disease mostly settles on the upper trunk and may spread towards the arms and abdomen. It usually causes no complaints, although mild itching may sometimes occur. Scaling is seen on light brown or café-au-lait patches of varying size.
Fine scaling is noticeable on the lesions, especially those that are yellow or café-au-lait in colour. If the surface of the lesions is scraped with a hard object, the scales can be seen to fall off; this is a characteristic feature of pityriasis versicolor and is called the ‘wood-shavings sign’ or “scraping sign”. This is because the fungi growing in the stratum corneum layer of the skin cause the horny cells there to loosen. On Wood’s lamp examination a pale yellow fluorescence is observed. Sometimes the Malassezia organisms causing the disease break down the melanin in the skin and create white patches; this picture is called “pityriasis versicolor alba”.
Naevus Depigmentosus
Naevus depigmentosus, or naevus achromicus, is a skin problem in which certain areas of the skin are hypopigmented or depigmented. The lesions of this skin disorder appear as non-raised, light-coloured or white patches that are present at birth or appear early in life; they are not progressive and do not spread or resolve with age. Unlike vitiligo, a multifactorial disease involving destruction of epidermal melanocytes, and albinism, which results from disorders of melanocyte development or differentiation, naevus depigmentosus involves reduced melanin production by melanocytes. It can also be confused with a similar-looking skin disorder called naevus anaemicus, but unlike naevus anaemicus it does produce redness after rubbing. Naevus depigmentosus cannot be cured, but it can be concealed with the use of suitable cosmetics.
The white or hypopigmented lesions do not enlarge, although they usually grow in proportion with the child’s growth. They are usually confined to the trunk and the upper arms and legs. The distribution may appear leaf-like, linear or segmental. In about 19% of people these lesions are seen at birth.
There is no pattern of inheritance in naevus depigmentosus, so it is not known whether this skin disorder is a dominant or recessive trait. Most newborns with naevus depigmentosus show no signs until they are babies or toddlers. The main pathology of naevus depigmentosus is reduced melanin production; therefore localised albinism is not an appropriate description, because albinism involves problems with the development or differentiation of melanocytes, the melanin-producing cells responsible for pigmentation.
To diagnose naevus depigmentosus, dermatologists use several criteria, such as the presence of hypopigmented or white lesions from birth or an early age, no change in distribution throughout life, no sensory disturbance in the affected area, and no hyperpigmentation surrounding the affected area. Although some patients show seizures, intellectual disability, sensitive skin and fair hair, associated symptoms are very rare. Because patients have reduced melanin and are more prone to sunburn, it is best for them always to use sunscreen or sun-protection products on the affected areas. Probably the best way to cope with naevus depigmentosus lesions, especially for self-conscious individuals, is to use cosmetics to cover the affected areas. Laser treatment or removal of the lesion can only be performed if a small area of skin is involved; another treatment option, melanocyte-keratinocyte transplantation (MKTP), has limited success.
Hypomelanosis of Ito
Hypomelanosis of Ito was described in 1952 by the Japanese dermatologist Minor Ito as a rare neurocutaneous syndrome under the name “Incontinentia Pigmenti Achromians”. Hypomelanosis of Ito is a neurocutaneous disease with characteristic cutaneous hypopigmentation distributed along the Blaschko lines, often accompanied by neurological, musculoskeletal, ocular, hair and/or chromosomal abnormalities.
Albinism
Albinism is a hereditary group of disorders of melanin biosynthesis characterised by a generalised reduction in the pigmentation of the eyes, skin and hair. Although albinism varies considerably around the world, it occurs in approximately 1 in 17,000 births.
It can be divided into two types according to the tissues involved. In ocular albinism (OA) only the eyes are involved, whereas in oculocutaneous albinism (OCA) the eyes, hair and skin are affected.
In albinism the changes in the eye are specific and of diagnostic importance. All types of OCA and OA involve varying degrees of nystagmus, light sensitivity (photophobia), reduced pigmentation (hypopigmentation) of the iris and retinal epithelium, foveal hypoplasia, refractive errors, reduced visual acuity and sometimes impaired colour vision. The most fundamental change in albinism is seen in the optic system. Misrouting of the optic nerves, with excessive crossing at the optic chiasm, is a characteristic finding. It results in squint and reduced stereoscopic vision. In addition, reduced visual acuity and the presence of ocular nystagmus in all types of OCA are features that distinguish OCA from other forms of congenital hypopigmentation.
Subtypes of OCA and OA have been described. The absence of pigment varies among these subtypes. In the intermediate forms (OCA1B, OCA2, OCA3 and OCA4) some pigment accumulates over time, whereas the OCA1A form is the most severe type, with a complete absence of melanin production throughout life.
OCA 1: caused by mutations in the tyrosinase gene (TYR) on chromosome 11q14.3. It is inherited in an autosomal recessive manner.
OCA 1A: the most severe form, in which the tyrosinase enzyme is completely absent. The hair, eyelashes, eyebrows and skin are white, and the irises are light-coloured and always fully translucent. Visual acuity is markedly reduced and photophobia is severe. No pigment develops, and amelanotic naevi may be present. The symptoms do not change with age.
OCA 1B: tyrosinase activity is reduced. Over the years the patient darkens somewhat (yellow-mutant albinism).
OCA TS (temperature-sensitive): tyrosinase can function below 35 degrees. In this case some pigmentation is seen in later life in the cooler acral areas.
OCA 2: there is a P gene mutation, and the responsible gene is located on chromosome 15q11.2-q12. Pigmentation can vary from normal to none; newborns almost always have pigmented hair. Naevi and freckles are common. Iris colour varies, and the pink eyes seen in OCA1A are usually absent. Visual acuity is usually better than in OCA1.
OCA 3 (rufous albinism): there is a mutation in TRP-1 (tyrosinase-related protein 1). It has been described only in people of Black African descent, who have red hair and reddish-brown skin.
OCA 4: a newly described type of OCA, for which a gene encoding a membrane-associated transporter protein is responsible. However, its function has not yet been fully clarified. OCA4 may be clinically indistinguishable from OCA2.
OA: shows an X-linked pattern of inheritance. Female carriers have a “mud-splattered” appearance of the fundus at the back of the eye. Diagnosis is made by observing macromelanosomes in a skin biopsy from patients.
In OCA, the diagnosis is made from the characteristic ocular symptoms together with the clinical findings of hypopigmentation of the hair and skin. On pathological examination, the number of melanocytes in the epidermis and hair follicles is normal. The melanin content of the melanocytes and neighbouring keratinocytes is reduced or absent.
OCA is one of the diseases for which prenatal diagnosis during pregnancy is also possible. Today prenatal diagnosis of OCA is possible using DNA obtained by chorionic villus sampling at weeks 10–12 of pregnancy or by amniocentesis in later weeks.
There is no primary treatment for albinism. Photosensitivity and skin cancers must always be kept in mind, so patients should be advised to use sunscreens and sunglasses.
Sun spots (melasma)
Sun spots (melasma) are non-congenital brown patches usually seen on the face. They are more common in women than in men. On the face they are usually bilateral and symmetrical. Histologically, melasma is divided into superficial, deep and mixed types. Superficial melasma responds better to treatment. Sun exposure, pregnancy, hormone treatments and contraceptive pills trigger melasma.
Melasma is a very common problem. Its rate is high in young women. It occurs more often in people with darker skin. Its cause is unknown. Thyroid diseases are often seen in people with melasma. However, no definite link has been established between melasma and thyroid diseases.
Melasma on the face is usually seen in sun-exposed areas such as the upper lip, cheeks and forehead. Rarely it may occur on the chin and forearms. It presents as dark, irregularly bordered patches that are not raised above the skin. Deep melasma may have a blue-grey tint and mixed melasma a brown-grey tint. Melasma usually tends to darken in the summer months and after sunbed use.
A Wood’s lamp (ultraviolet lamp) can determine the depth of melasma. If the patch becomes more prominent under the Wood’s lamp it is superficial; if there is no change it is deep. Patients with melasma need to use broad-spectrum sunscreen continuously and avoid artificial light sources such as sunbeds. Hormone medicines and contraceptive pills should not be used unless absolutely necessary.
How does melasma on the face go away?
Melasma Treatment:
In the treatment of melasma, topical pigmentation creams, oral antioxidant tablets, laser treatments and chemical peels can be used alone or in combination. People with melasma should use sun protection products of at least SPF 30 regularly, summer and winter, every 4 hours during the day. Even when using sunscreen, they should avoid too much direct sun exposure, take care to stay in the shade and wear a hat and sunglasses. Care must be taken in the treatment of melasma. Very aggressive treatments should be avoided when treating pigmentation, as they can trigger it.
Solutions for melasma
- Laser treatments
- Skin-lightening creams
- Chemical peeling and enzymatic peeling
- Mesotherapy – PRP
Laser treatment of melasma:
The lasers used in pigmentation treatment work by resurfacing the skin or by destroying the pigment cells (melanocytes).
- Intense pulsed light (IPL)
- Pulsed dye laser (510 nm)
- Q-switched Nd:YAG laser (1064 nm)
- Fractional lasers
Laser treatments can be used for this type of pigmentation. However, care must be taken. An increase in pigmentation, or recurrence within a short time, is seen.
Creams for melasma:
In melasma treatment, medicines are generally used as creams. The substances used in pigmentation treatment work through different mechanisms. The main mechanisms of action of pigmentation medicines are:
- They suppress the tyrosinase enzyme involved in the formation of pigment (melanin)
- They prevent the transfer of pigment (melanin) from melanocytes to other cells
- They damage the pigment cells (melanocytes)
- They renew the skin (antioxidant creams and tablets, retinoids, fruit acids)
Chemical peeling for melasma on the face:
Fruit acid peels and TCA peels can be used in pigmentation treatment. An average of 5 sessions at 1–4 week intervals is required. Chemical peeling can be effective by renewing the skin and increasing the absorption of the pigmentation medicines used. Chemical peeling should be applied carefully in people with darker skin. Chemical peels that go too deep can increase pigmentation. For this reason chemical peeling must be applied very carefully, without being aggressive for the sake of peeling the skin. It can backfire.
Mesotherapy and PRP for melasma on the face:
In pigmentation treatment, injecting small amounts of substances such as vitamin C, glutathione, tranexamic acid and pyruvic acid under the skin of the pigmented area can lighten the colour. It is not a standard method and can be considered when other methods have not been successful.
The plasma (PRP) method can be successful in pigmentation treatment when combined with laser. In general, the pigmented areas of the skin are the areas most damaged by the sun. For pigmentation treatment, the PRP method requires 3–4 sessions at 2–4 week intervals. Using the PRP method, which intensively repairs the skin, together with pigmentation lasers can make the pigmentation disappear or fade. It also contributes to skin rejuvenation.
Erythema Dyschromicum Perstans
Ashy dermatosis was first described in El Salvador in 1957 by Ramirez under the name “dermatosis cenicienta”; in 1961 Convit and colleagues renamed the disease “erythema dyschromicum perstans”. Although many cases have been reported from Central and South America, it is rare in other parts of the world. Erythema dyschromicum perstans (EDP), a progressive pigmentation disorder characterised by asymptomatic purplish to ash-grey macules that are usually resistant to various treatments and, as the face is often also involved, cause a cosmetically very distressing appearance, mostly begins in the first and second decades. Both sexes are affected equally. Around the macules, which vary in size, shape and intensity, a 1–2 mm wide, slightly raised peripheral erythema may be seen in the acute phase, sometimes becoming more prominent with heat and sweating. After a few months, however, this erythema disappears and is sometimes replaced by a pale ring, lighter than normal skin, that makes the ash-coloured lesion more striking. In the chronic phase usually only purplish-grey macules are observed. Because of its rarity, a case of EDP is presented in this report and the literature on the subject is reviewed.
A 12-year-old girl from a rural area was brought in with a complaint of dark, non-itchy patches that had been appearing all over her body except the hands and feet for about two years; apart from constant sun exposure and occasional exposure to agricultural pesticides, there was nothing notable in her personal or family history. Her history revealed that she had previously used hydroquinone and sunscreen creams intermittently for these patches, and chloroquine continuously for six months, without benefit. On physical examination and laboratory tests all findings were normal apart from mild microcytic hypochromic anaemia. Dermatological examination showed purplish-grey macules 1–2 cm in size with no particular shape, more dense on the trunk, face and proximal parts of the limbs, and becoming darker and tending to merge in the intertriginous areas. Darier’s sign was negative on the lesions. The lesions became gradually sparser towards the distal parts of the limbs, and no pigmented macules were found on the hands and feet. The nails, scalp and mucosae appeared normal. On the face, band-like perioral and periorbital hyperpigmentation was observed, together with a few scattered individual pigmented macules on the cheeks and forehead.
Histopathological examination (haematoxylin-eosin) of a 4 mm punch biopsy taken from an ash-coloured macule in the gluteal region showed sparse, thinned and elongated rete ridges, intracytoplasmic vacuoles in some keratinocytes of the stratum spinosum and focal minimal vacuolisation of the basal layer, a mononuclear cell infiltrate that was sparse in the papillary dermis and concentrated perivascularly in the mid-dermis, and melanin pigment both within melanophages and free. Toluidine blue staining showed very few mast cells. With a diagnosis of chronic-phase EDP based on the clinical and histological findings, the patient was started on dapsone (100 mg/day) and oral iron for iron-deficiency anaemia. As the lesions did not improve after two months of treatment, clofazimine (100 mg/day) was started, but no good response was obtained despite three months of use.
Ashy dermatosis or EDP is a pigmentary disease of unknown aetiology and prevention that, apart from a few cases, does not respond to treatment. Oral radiocontrast agents, pesticides such as chlorothalonil and fucilade, ingestion of ammonium nitrate, Trichuris trichiura infestation, thyroid diseases and atopy have been implicated in its aetiology. Cases of EDP developing before and after lichen planus have been reported, and it has been suggested that the two diseases may be related. The results of some histopathological and immunofluorescence studies also support this view. In an immunopathological study of EDP, both CD4/helper and CD8/suppressor T lymphocytes and IgG-positive colloid bodies were detected in the dermis, similar to lichen planus.
Henderson and colleagues reported a patient with vitiligo who simultaneously had hyperpigmented macules typical of EDP. On the other hand, an immunohistochemical study of vitiligo and EDP found, in the early stages of both diseases, selective cytotoxic T cells in the epidermis, Ia antigen positivity in dermal and epidermal dendritic cells and in most lymphoid cells, an increase in Langerhans cells in some cases, and interleukin-2 receptor (CD25, TAC) expression in dermal lymphocytes, concluding that immunological factors may contribute to the dyschromia in both diseases. Histopathological examinations of patients with EDP showed, in active lesions, findings of lichenoid dermatitis characterised by basal cell vacuolisation and CD8 suppressor T lymphocyte infiltration, and on direct immunofluorescence cytoid bodies showing IgM and C4 deposits and fibrinogen at the dermo-epidermal junction; in old lesions only fibrinogen, pigment incontinence in the basal layer, a mild perivascular lymphohistiocytic infiltrate in the upper dermis and melanin-laden macrophages were seen, and it was suggested that basal cell apoptosis is responsible for the post-inflammatory hyperpigmentation seen in EDP and other dermatoses. In another study, ICAM-1 (intercellular adhesion molecule-1) and the class II major histocompatibility antigen HLA-DR were positive in basal cells in EDP, and CD36 in the stratum spinosum and granulosum, whereas these molecules were found to be negative after 3 months of treatment with clofazimine.
In the differential diagnosis of EDP, multiple fixed drug eruptions, chloasma, Riehl’s melanosis, pinta, argyria, leprosy, maculae ceruleae, annular and atrophic lichen planus, haemochromatosis, Addison’s disease, graft-versus-host disease and, in very dark-skinned people, cutaneous mastocytoses such as urticaria pigmentosa and telangiectasia macularis eruptiva perstans should be considered. In our case, macular urticaria pigmentosa was ruled out by the low number of mast cells on toluidine blue examination of the biopsy material and the negative Darier’s sign, and lichen planus pigmentosus (actinic lichen planus) by the absence of severe vacuolar degeneration of the basal layer and a dense band-like infiltrate.
Over time, EDP lesions may progress and merge, taking on a polycyclic and annular character. In our patient, lesions that merged into bands were seen particularly in the periorbital and perioral areas, but images of the face could not be taken because the patient and her family did not give permission. In a recently published report, Combemale and colleagues described a case of EDP from North Africa with dyschromic lesions showing both hyper- and hypopigmentation, expanding centrifugally and at times showing an active papular border, and suggested that the EDP described by Convit and the ashy dermatosis reported by Ramirez are in fact different entities. However, both terms are still used synonymously to describe the same clinical entity.
Doxycycline, corticosteroids, sunscreens, chemical peeling, chloroquine, isoniazid and vitamins are used in treatment, but the response is generally poor. Although cases of spontaneous improvement have rarely been reported, the dark pigmented patches are generally resistant to treatment. A small number of cases respond well to griseofulvin and dapsone. Clofazimine is the most successful of the treatments tried. With clofazimine, which improves the cellular immune response by altering the CD4/CD8 ratio, used at a dose of 100 mg per day for 3 months, excellent results were obtained in seven of eight cases. In our case, who had previously used hydroquinone and chloroquine, no good response was obtained with dapsone or clofazimine either. Dapsone and clofazimine, which may be useful in treatment probably by correcting the immunological disturbances in the pathogenesis of EDP, appear to be ineffective in treating old lesions.
Carotenaemia
This is a harmless condition that occurs when blood levels of carotene, the orange pigment found in some vegetables, rise as a result of excessive consumption of these foods. Carotenaemia causes temporary yellowing of the skin, particularly on the palms and soles. Unlike jaundice, carotenaemia does not cause yellowing of the whites of the eyes.