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AlexanderSalava

Skin Colour Changes and Pigmentary Disorders

Essentials

  • Pigmentary disorders may cause locally or generally darker (hyperpigmentation) or lighter (hypopigmentation) skin.
  • Even though the skin lesions are usually asymptomatic, they may affect the patient's quality of life significantly (cosmetic disturbance and psychosocial problems).
  • Drugs and certain systemic diseases may cause generalized or local changes of skin colour.

Pathogenesis

  • Changes in skin colour are usually due to increased or decreased amounts of melanin pigment and/or of melanocytes (hyperpigmentation or hypopigmentation, respectively).
  • Chronic abrasion may lead to thickening of the epidermis, causing visible hyperpigmentation (e.g. calluses, mechanical abrasion, neurodermatitis).
  • In generalized pigmentary disorders the cause may be hormonal (e.g. melanocyte stimulation caused by high levels of melanocyte-stimulating hormone [MSH] in patients with hypocortisolism, or melasma during pregnancy).
  • Skin colour may also change due to metabolic products accumulating in the skin (such as bilirubin in patients with jaundice or iron in patients with haemochromatosis).

Diagnosis

  • The diagnosis is usually based on the patient history and clinical picture.
  • What colour are the skin lesions? Is the pigmentary disorder local or generalized? How have the skin lesions developed? Does their location change?
  • Does the patient have any history of diagnosed skin disorders, such as vitiligo or atopic eczema?
  • Has the patient developed skin reactions in association with sun exposure? Are the locations typical for photodermatitis (face, neck, chest, arms)?
  • Is it an acquired or a congenital disorder? Is there any family history of pigmentary disorders?
  • Hormonal changes, oral contraceptives or pregnancy
  • Oral medication or topical skin treatment, including natural drugs and treatments, sunscreen ointments and cosmetics
  • Examine the whole skin and mucosa, including the palms and soles of the feet.

Localized darkening of the skin (hyperpigmentation)

Most common causes

  • Pityriasis versicolor Pityriasis Versicolor (picture 1)
    • Reddish brown, slightly scaly patches
    • Most often on the upper trunk, neck and proximal parts of the limbs
    • N.B.: Hypopigmentation may also occur (see below)
  • Melasma (picture 2)
    • Clearly defined, light brown, hyperpigmented patches
    • In areas of the skin exposed to the sun, usually the face
    • May appear during pregnancy or after using hormonal contraception.
  • Postinflammatory hyperpigmentation: when healing, many skin disorders may leave slowly fading dark patches; eczemas, psoriasis Psoriasis, purpura Easy Bruising, Petechiae and Ecchymoses, lichen ruber planus Lichen Planus, for instance.
  • Physiological changes in pigmentation during pregnancy Skin Problems during Pregnancy
  • Café au lait spots
    • Clearly defined light brown patches on the trunk or the limbs (picture 3)
    • Single patches are quite common; numerous patches in a child or adolescent may suggest neurofibromatosis.

Less common causes

  • Diabetic dermopathy: clearly defined light brown patches on both legs
  • Acanthosis nigricans (pictures 4 5)
    • Dark discolouration of armpits and groin
    • May cause warty thickening of the skin.
    • May be idiopathic but is usually due to obesity or type 2 diabetes.
    • When occurring as a new symptom in an adult may represent a paraneoplastic phenomenon.
  • Allergic contact eczema (Riehl melanosis) Allergic Contact Dermatitis
    • In areas of skin exposed to sun allergic contact eczema may cause patchy hyperpigmentation.
    • This usually represents contact allergy to cosmetic ingredients (such as perfumes or preservatives).
  • Photocontact dermatitis (Berloque dermatitis) Photodermatitis
    • May be caused by perfumes, sunscreen chemicals, analgesic gels or ointments.
    • In addition, some plants may cause what is called phytophotodermatitis.
  • Drug-induced photodermatitis Photodermatitis: diffuse hyperpigmentation may develop in light-exposed areas after eczema.
  • Fixed drug eruption (erythema fixum; picture 6)
    • Appears always in the same place after drug intake.
    • May leave a slowly fading dark patch on healing.
  • Becker's naevus (picture 7)
    • Usually appears in childhood but may become darker in teen years or early adulthood.
    • Most commonly occurs in the shoulder or flank area.
  • Poikiloderma of Civatte (picture 8)
    • Reddish brown, reticular hyperpigmentation, skin atrophy and telangiectasia on the cheeks and neck
    • In adults and elderly people
    • Cause unclear; considered to be due to chronic photodamage.
  • Erythromelanosis follicularis faciei
    • Reddish brown, patchy hyperpigmentation, telangiectasia and follicular papules on both cheeks and temples and below the eyes
    • In adolescence or early adulthood; more common in men
    • Probably hereditary
  • Erythema dyschromicum perstans (ashy dermatosis)
    • Oval or round greyish brown hyperpigmented patches appear on the trunk, more rarely on the limbs or the face.
    • Cause unclear
    • Has a chronic course

Localized lightening of the skin (hypopigmentation)

Most common causes

  • Pityriasis versicolor Pityriasis Versicolor (picture 9)
    • May also cause light, slightly scaly patches on the skin.
  • Vitiligo Vitiligo (picture 10)
    • Asymptomatic, clearly defined white patches usually symmetrically on the limbs, on the trunk, in the navel area and on the nipples, on the face around the mouth and eyes
  • Postinflammatory hypopigmentation: when healing, many skin disorders, such as psoriasis and atopic eczema, may leave slowly fading, white patches.
  • Pityriasis alba
    • Slightly scaly, white patches predominantly on the face and upper limbs
    • Cause unknown; it may represent postinflammatory hypopigmentation and be associated with atopic eczema.
    • Has a chronic course

Less common causes

  • Depigmented naevi (halo naevi): a white, annular patch develops around a naevus.
  • Idiopathic guttate hypomelanosis
    • Small white patches on the limbs in photodamaged skin in the elderly
    • Clinical picture resembling confetti
  • Some topical drugs may cause white patches (retinoids, benzoyl peroxide and azelaic acid used for the treatment of acne, and topical and injectable glucocorticoids, for instance).
  • Progressive macular hypomelanosis
    • Asymptomatic white patches on the trunk, neck and proximal limbs, often merging in the midline
    • Usually in people with dark skin
    • Cause unclear
  • Piebaldism
    • Pigmentary disorder with autosomal dominant inheritance
    • White patches, most commonly on the scalp or facial area from birth
    • Hair or eyebrows grow white in the affected areas (poliosis).
    • May also occur on the trunk and limbs.
    • The location of the lesions does not change.
  • White patches in patients with tuberous sclerosis: white (ash-leaf) spots from birth on the trunk and limbs; additionally facial fibromas and epilepsy
  • Leprosy (tuberculoid leprosy) Bacterial Diseases in Warm Climates
    • White patches predominantly on the limbs, with reduced skin sensation and reduced sweating
    • May occur in immigrants

Generalized (diffuse) pigmentary disorders

  • Usually diffuse, symmetric changes in skin colour.
  • If there is an underlying systemic disease, the pigmentary disorder appears as a new symptom in a patient with other findings, as well.

Hyperpigmentation

  • Drug-induced diffuse hyperpigmentation
    • Most commonly caused by antimalarial drugs, hydroxychloroquine, amiodarone, minocycline, anticancer drugs, chlorpromazine, tricyclic antidepressants or antiepileptic drugs
  • Addison's disease Addison's Disease and other Conditions Inducing Hypocortisolism
    • Diffuse darkening of the skin, particularly in the creases on the palms and on the soles of the feet
    • Gingival and oral mucosal hyperpigmentation as a new symptom
  • Vitamin B12 deficiency Megaloblastic Anaemia
    • Diffuse darkening of the skin and Addison's disease-like clinical symptoms and findings
    • Other symptoms include anaemia, neurological symptoms and atrophic glossitis
  • Acromegaly due to a pituitary tumour Pituitary Tumours and Cushing's syndrome Cushing's Syndrome: diffuse darkening of the skin due to melanocyte-stimulating hormone (MSH) secretion
  • Haemochromatosis Haemochromatosis
    • Diffuse bronze or slate grey darkening of the skin, 'bronze diabetes'
    • Predominantly on areas exposed to light: the face, neck, upper limbs
    • Patchy hyperpigmentation of the mucosa
  • Porphyria cutanea tarda Porphyrias (picture 11)
    • Melasma-type patchy hyperpigmentation of the face
  • Chronic renal failure Treatment of Chronic Renal Failure
    • Diffuse yellowish brown hyperpigmentation of the skin
  • Liver disorders (cirrhosis of the liver Cirrhosis of the Liver, liver cancer and metastases, primary biliary cholangitis Primary Biliary Cholangitis)

Hypopigmentation

  • Hypothyroidism Hypothyroidism
    • The skin may be pale, dry and scaly.
  • Pituitary failure (hypopituitarism Pituitary Tumours)
    • Diffuse lightening of the skin
  • Albinism, other genetic syndromes and metabolic disorders: diffuse hypopigmentation usually since birth

Jaundice

  • Jaundice A Patient with Jaundice (Icterus)
    • Cholestasis, liver disorders and liver metastases may cause also diffuse yellowish brown darkening of the skin.
  • Hypercarotenaemia
    • Excessive intake of carrots or other food or natural products containing carotenes
    • Diabetes, liver or kidney disease, hypothyroidism
  • Elastosis (picture 12)
    • Chronic exposure to sunlight, particularly on the face of elderly people
    • Deep furrows and yellowish skin tone
  • Darkening or yellowing caused by local factors
    • In association with some topical treatments (e.g. self-tanning creams), the skin colour may change.
    • Terra firma-forme dermatosis
      • Usually in young people, brown, on the surface wart-like or plaque-like areas of skin, typically on the neck, torso or navel area
    • Dermatitis neglecta
      • Caused by poor mechanical washing of the skin and neglect of hygiene
      • Localized dark patches on the skin, with flaky dandruff on them, may appear as wart-like plaques, typically on the torso, genital area and around surgical or plaster areas.

Workup

  • Further tests are rarely needed; the clinical picture is decisive.
  • In pityriasis versicolor and seborrhoeic eczema, Malassezia yeast may be seen on microscopy (native fungal examination) but fungal culture may still be negative. The diagnosis is based on clinical features.
  • Histological examination of a skin biopsy specimen may be helpful if a specific skin disorder is suspected. The biopsy specimen should be taken at an active stage of the disease.
  • Epicutaneous and/or photopatch tests may be indicated if allergic contact dermatitis is suspected.
  • If a pigmentary disorder due to an internal disease is suspected, targeted, stepped laboratory tests should be performed, as necessary.

Treatment

Hyperpigmentation

  • Treatment of pityriasis versicolor Pityriasis Versicolor
  • Sun protection and use of a suitable sunscreen ointment often help.
  • Causal treatment (visceral diseases; avoidance of causative factor in allergic contact dermatitis, photodermatitis, drug-induced pigmentary disorders)
  • In the case of postinflammatory pigmentary disorders, treatment of the causative skin disease. The response for the actual pigmentary disorder is often poor but the skin tone often evens out within 6-12 months.
  • For local hyperpigmentation (melasma, postinflammatory hyperpigmentation) there is evidence of the efficacy of (2-4%) hydroquinone ointments Interventions for Melasma.
    • A combination cream containing hydroquinone, tretinoin and dexamethasone (Pigmanorm®) is available with special permit for compassionate use. A thin layer of the ointment is applied once daily, at night, in courses of 1-3 months until the skin area becomes lighter.
    • Mequinol 10% cream (Leucodinine B 10%, may require a special permit, substance belongs to hydroquinones). Usually used twice a day for local hyperpigmentation patches. When improvement is noticeable (within a few weeks), the application frequency can be reduced.
  • There is also some evidence for the efficacy of topical treatment with azelaic acid or retinoids (e.g. 0.025% or 0.05% tretinoin ointment).
    • For example, once daily, at night, in courses of 1-3 months, depending on response
  • In severe cases, topical treatment has been combined with glycolic acid peeling and laser treatments (possibly available through private health care only).

Hypopigmentation

  • Treatment of pityriasis versicolor Pityriasis Versicolor
  • Treatment of vitiligo Vitiligo
  • For hypopigmentation of other causes, there are no evidence-based treatments available.
  • Covering treatment and makeup

Specialist consultation

  • For severe pigmentary disorders or ones causing significant psychosocial suffering, consultation of a dermatologist should be considered.

    References

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    • Soundararajan V, Charny JW, Bain MA, et al. The color of skin: orange diseases of the skin, nails, and mucosa. Clin Dermatol 2019;37(5):520-527 [PubMed]
    • Logan IT, Logan RA. The color of skin: yellow diseases of the skin, nails, and mucosa. Clin Dermatol 2019;37(5):580-590 [PubMed]
    • Kutlubay Z, Cesur SK, Aşkın Ö, et al. The color of skin: brown diseases of the skin, nails, and mucosa. Clin Dermatol 2019;37(5):487-506 [PubMed]
    • Ezzedine K, Whitton M, Pinart M. Interventions for Vitiligo. JAMA 2016;316(16):1708-1709. [PubMed]
    • Whitton M, Pinart M, Batchelor JM, et al. Evidence-based management of vitiligo: summary of a Cochrane systematic review. Br J Dermatol 2016;174(5):962-9. [PubMed]
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    • Hu SW, Bigby M. Pityriasis versicolor: a systematic review of interventions. Arch Dermatol 2010;146(10):1132-40. [PubMed]
    • Kang HY, Valerio L, Bahadoran P, et al. The role of topical retinoids in the treatment of pigmentary disorders: an evidence-based review. Am J Clin Dermatol 2009;10(4):251-60. [PubMed]