Skin Pigmentation Disorders

Pigmentation means coloring. Skin pigmentation disorders affect the color of your skin. Your skin gets its color from a pigment called melanin. Special cells in the skin make melanin. When these cells become damaged or unhealthy, it affects melanin production. Some pigmentation disorders affect just patches of skin. Others affect your entire body.

If your body makes too much melanin, your skin gets darker. Pregnancy, Addison's disease, and sun exposure all can make your skin darker. If your body makes too little melanin, your skin gets lighter. Vitiligo is a condition that causes patches of light skin. Albinism is a genetic condition affecting a person's skin. A person with albinism may have no color, lighter than normal skin color, or patchy missing skin color. Infections, blisters and burns can also cause lighter skin.

Different Conditions

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Acanthosis nigricans

Benign acanthosis nigricans is a thickening and pigmentation affecting predominantly flexural skin, especially the neck, axillae and groins. It is thought to be due to high concentrations of insulin binding to insulin-like growth factor receptors, with resultant keratinocyte proliferation. It is strongly associated with insulin resistance and obesity. Type 2 diabetes and the metabolic syndrome are commonly associated.

Abbreviated Terms

  • Acanthosis nigricans associated with Type II diabetes and obesity
  • Related ICD 10 COde
    ICD 10 Code L83

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    Acquired hypermelanosis

    Melanin pigmentation of the skin resulting from preceding cutaneous inflammation, particularly when this is centred on the dermo-epidermal junction as in lichen planus. Damage to melanocytes results in release of melanin into the dermis (pigmentary incontinence).

    Also Known As

  • Postinflammatory hyperpigmentation
  • Related ICD 10 COde
    ICD 10 Code L81.0

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    Signs And Symptoms

  • Depigmentation (morphologic abnormality)
  • Related ICD 10 COde
    ICD 10 Code L81.5

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    Acquired melanotic macules or lentigines

    The presence of multiple ephelides (ephelis = freckle) as is commonly seen in sun-exposed skin of individuals with phototype I (sun-sensitive) skin. They occur as a profusion of light brown macules, particularly on the face and upper extremities, and become more prominent after sun exposure. In contrast to lenitigines there is no keratinocyte proliferation histologically.

    Organ Affected

    Subcutaneous tissue structure (body structure)|Skin structure (body structure)|Entire skin (body structure)

    Also Known As

  • Ephelides
  • Related ICD 10 COde
    ICD 10 Code L81.2

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    Acquired mixed hyper- and hypomelanotic disorders of skin pigmentation

    Dyschromatosis from chronic arsenic toxicity presents as hypermelanotic and hypomelanotic symmetrically distirbuted freckle or raindrop-like macules on the torso and limbs. These may be preceded by diffuse hyperpigmented patches in the intertriginous, genital, and acral areas.
    Related ICD 10 COde
    ICD 10 Code L81.9

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    Albinism or other specified genetically-determined hypomelanotic disorders

    Hypomelanotic streaks in Blaschko line distribution as a result of a somatic mutation. It may be associated with eye, skeletal and CNS abnormalities.
    Related ICD 10 COde
    ICD 10 Code E70.3

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    Also Known As

  • Progressive pigmented purpura of Schamberg
  • Schamberg disease
  • Related ICD 10 COde
    ICD 10 Code L81.7

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    Chronic effects of ultraviolet radiation on the skin

    Pigmentation, telangiectasia and atrophy due to repeated contact with photoactive fragrances. This is most commonly seen on the sides of the neck in women as a result of perfume use.
    Related ICD 10 COde
    ICD 10 Code L57.3

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    Diffuse epidermal hyperkeratosis and acanthosis

    Acanthosis nigricans is characterized by dark, thick, velvety skin in body folds and creases. It is most commonly encountered in association with obesity and type 2 diabetes though may be a component of a number of genetic syndromes. Hyperinsulinaemia and insulin resistance are important underlying factors. Acanthosis nigricans may rarely be due to underlying malignancy (paraneoplastic acanthosis nigricans).

    Signs And Symptoms

  • Hyperpigmentation of skin (disorder)
  • Hyperpigmentation (morphologic abnormality)
  • Organ Affected

    Subcutaneous tissue structure (body structure)|Skin structure (body structure)|Entire skin (body structure)
    Related ICD 10 COde
    ICD 10 Code L83

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    Disorders of skin colour

    Increased melanin pigmentation of the skin resulting from disease or from other stimuli including ultraviolet radiation and hormones.
    Related ICD 10 COde
    ICD 10 Code L81.9

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    Disorders of the epidermis and epidermal appendages

    This group includes not only abnormalities of melanin pigmentation (e.g. vitiligo and melasma) but also skin colour changes due to other pigments (e.g. carotenaemia and argyria).
    Related ICD 10 COde
    ICD 10 Code L81.9

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    Ectodermal dysplasia syndromes

    A variant of hypomelanotic pigmentary mosaicism characterized by extensive hypomelanotic streaks and whorls.

    Where It Occurs

    Skin System (Integumentary System)
    Related ICD 10 COde
    ICD 10 Code E70.3

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    Endogenous non-melanin pigmentation

    Dermal haemosiderin deposition causes a yellowish-brown or bronze discolouration of the skin. The deposition may be focal as seen following repeated extravasation of red blood cells (e.g. in association with venous hypertension or chronic vasculitis) or from generalized iron overload (e.g. haemochromatosis). Haemosiderin may stimulate melanogenesis and thus the colour is due to variable proportions of haemosiderin and melanin.
    Related ICD 10 COde
    ICD 10 Code L81.9

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    Genetic disorders of skin pigmentation

    A large group of heritable disorders in which cutaneous melanin production is reduced or absent, mainly as the result of defects in enzymes required for normal melanin biosynthesis.

    Signs And Symptoms

  • Albinism (disorder)
  • Related ICD 10 COde
    ICD 10 Code E70.3

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    Genital melanosis

    Discrete circumscribed area of macular hypermelanosis affecting the glans penis or the shaft of the penis. These are much commoner than melanoma of the penis but may be clinically suspected to be melanoma and thus require biopsy. By definition the cause is unknown.

    Where It Occurs

    Skin System (Integumentary System)|Male Genital System

    Organ Affected

    Skin of penis
    Related ICD 10 COde
    ICD 10 Code L81.9

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    Haemosiderin pigmentation of skin

    Haemosiderosis of the lower legs resulting from recurrent extravasation of red blood cells into the dermis in people with venous hypertension.
    Related ICD 10 COde
    ICD 10 Code L81.9

    ----------------------

    Related ICD 10 COde
    ICD 10 Code E70.3

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    Hypomelanotic pigmentary mosaicism

    Phylloid hypomelanosis is a rare neurocutaneous syndrome characterized by a pattern of hypopigmentation consisting of leaflike or oblong macules reminiscent of floral ornaments. Associated extracutaneous anomalies include cerebral, ocular, and skeletal defects.
    Related ICD 10 COde
    ICD 10 Code E70.3

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    Miscellaneous specific cutaneous signs of internal malignancy

    Generalized hypermelanosis due to secretion of ACTH or similar peptides by malignant tumours, especially bronchial carcinoma and pulmonary carcinoid.

    Where It Occurs

    Skin System (Integumentary System)
    Related ICD 10 COde
    ICD 10 Code L81.9

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    Mucosal melanosis

    Laugier-Hunziker melanosis is an acquired pigmentary disorder characterized by variable numbers of well-defined brown to black lentigines on the lower lip, buccal mucosa and hard palate, frequently in association with longitudinal melanonychia and genital melanosis. It is not associated with gastrointestinal polyposis or other internal disorder.
    Related ICD 10 COde
    ICD 10 Code L81.9

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    Non-melanin pigmentation due to exogenous substances

    Pigmentation of the skin due to topically applied therapeutic agents such as anthralin and potassium permanganate.
    Related ICD 10 COde
    ICD 10 Code L81.9

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    Non-melanin pigmentation due to ingested or injected substance

    Carotenoderma results from excessive ingestion of carotene from foods such as oranges and carrots. Carotene contributes a yellow component to the colour of normal skin but when taken in excess may impart a diffuse yellow or orange-yellow tinge to the skin, especially on the palms and soles.
    Related ICD 10 COde
    ICD 10 Code L81.9

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    Related ICD 10 COde
    ICD 10 Code E70.3

    ----------------------

    Oculocutaneous albinism type 2

    Individuals with Brown-OCA2 may present with the classic OCA2 phenotype (yellow/blond hair and blue/hazel irides) except for more evident skin pigmentation. It is caused by mutations in the OCA2 (P) gene and occurs principally in Sub-Saharan Africa.
    Related ICD 10 COde
    ICD 10 Code E70.3

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    Oculocutaneous albinism

    Rare syndrome characterized by hypopigmentation of the skin, microphthalmia and cerebral abnormalities which result in spasticity and mental retardation.

    Also Known As

  • Cross syndrome
  • Related ICD 10 COde
    ICD 10 Code E70.3

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    Partial albinism

    This type, caused by mutations in MLPH or MYO5A genes, presents with hypopigmentation alone.

    Also Known As

  • GS3 - [Griscelli syndrome type 3] (MIM 609227)
  • Related ICD 10 COde
    ICD 10 Code E70.3

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    Physiological hypermelanosis

    Increased melanin pigmentation of the skin as a result of exposure to natural sunlight.
    Related ICD 10 COde
    ICD 10 Code L81.9

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    Pigmentary abnormalities of skin due to drug

    Discolouration of the skin due to deposition of pigments from drugs or their metabolites, usually after long-term use. Well-known causes include minocycline, mepacrine, clofazimine and amiodarone.
    Related ICD 10 COde
    ICD 10 Code L81.9

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    Primary haemophagocytic lymphohistiocytosis

    This syndrome is caused by mutations in the LYST gene. It consists of partial OCA, immunodeficiency, mild bleeding tendency and lymphoproliferative infiltration of bone marrow and reticuloendothelial system.

    Additional Information

    Ch???diak-Higashi's syndrome is a very rare recessive autosomal disease. Patients present with a combination of oculocutaneous albinism with decreased pigmentation, silvery-blond hair, hepatosplenomegaly, ganglionic hypertrophia and recurrent pyogenic cutaneo-respiratory infections. These signs result from functional anomalies of polynuclear cells, which contain large characteristic lysosomal inclusions, and from Natural Killer (NK) lymphocytes deficiency. Vital prognostic is very severe. The CHS gene was localised to the long arm of chromosome 1, 1q42.1-q42.2. An animal model of the disease, the beige mouse, allowed the discovery of a gene encoding LYST protein, whose function remains unknown.

    Also Known As

  • Chediak-Higashi-Steinbrinck syndrome
  • Chediak-Steinbrinck syndrome
  • B???guez C???sar syndrome
  • Steinbrinck anomaly
  • Related ICD 10 COde
    ICD 10 Code E70.3

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    Syndromes with multiple structural anomalies, not of environmental origin

    Autosomal dominant inherited syndrome consisting in congenital sensorineural hearing loss and hypomelanosis of skin, hair and irides.

    Also Known As

  • White forelock syndrome
  • Related ICD 10 COde
    ICD 10 Code E70.3

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    Syndromic acquired hypomelanosis

    A rare syndrome characterized by the development of unilateral retinal degeneration with ipsilateral facial vitiligo, poliosis and hearing loss.
    Related ICD 10 COde
    ICD 10 Code L81.9

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    Syndromic genetic deafness

    Tietz hypomelanosis - deafness syndrome is an autosomal dominant condition characterised by generalized hypomelanosis, blue eyes and profound sensorineural deafness. Affected family members gradually gain some pigmentation and have fair skin and blond to white hair as adults, with white eyebrows and eyelashes. The syndrome is due to a missense mutation in the basic region of the MITF gene.

    Additional Information

    Tietz syndrome is an autosomal dominant syndrome of hypopigmentation and deafness. Affected family members are born "snow white". They gradually gain some pigmentation and have fair skin and blond to white hair as adults with white eyebrows and eyelashes. All of them have blue eyes. Hearing loss is always bilateral, congenital, sensorineural and profound. The syndrome is due to a missense mutation in the basic region of the MITF gene.
    Related ICD 10 COde
    ICD 10 Code E70.3

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    Vasculitis or capillaritis involving the skin

    Capillaritis results from extravasation of red blood cells from leaky capillaries into the dermis and manifests initially as a finely stippled pink to purple purpura, most commonly affecting the lower limbs. As iron is released and converted into haemosiderin, the skin stains gold or brown. Various patterns of capillaritis have been described and given separate names depending on the distribution, time course, extent and degree of pigmentation and presence or otherwise of epidermal thickening. The underlying processes involved in all these variants is very similar and of unknown cause. Histology may show mild inflammation around capillaries but no vasculitis.

    Signs And Symptoms

  • Purpura
  • Haemosiderin pigmentation of skin
  • Where It Occurs

    Skin

    Organ Affected

    Skin

    Also Known As

  • Pigmented purpura
  • Purpura simplex
  • Related ICD 10 COde
    ICD 10 Code L81.7

    ----------------------

    Waardenburg syndrome

    This type is due to mutations in PAX3 gene and has the additional phenotype characteristic of dystopia canthorum.
    Related ICD 10 COde
    ICD 10 Code E70.3

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