What Causes Hair Heterochromia?

Hair heterochromia is a condition where hair on the same individual exhibits two or more distinct colors, often appearing as a localized patch of light hair contrasted against the natural color. This variation results from a disruption in the hair follicle’s ability to produce or distribute melanin in specific areas. The causes range from congenital defects in pigment cell development to acquired damage that occurs later in life.

Understanding Hair Color Variation

The color of a person’s hair depends on specialized pigment-producing cells called melanocytes, which reside in the hair follicle bulb. These cells produce melanin, a pigment transferred to the keratinocytes that make up the hair shaft. Hair color is determined by the relative amounts and types of melanin present, primarily eumelanin (black and brown tones) and pheomelanin (yellow and red tones).

Hair heterochromia is most frequently seen as poliosis, a localized decrease or complete absence of melanin in the hair shaft and root. Poliosis results in a patch of white or gray hair, commonly known as a white forelock, while the surrounding hair maintains its natural color. This condition reflects a failure or death of the melanocytes within the affected hair follicle. The underlying cause determines whether this localized depigmentation is present from birth or develops over time.

Inherited Conditions Affecting Pigmentation

Many cases of localized hair depigmentation are congenital, stemming from genetic mutations that disrupt the normal development and migration of melanocytes during embryonic development. These conditions are classified as neurocristopathies because melanocytes originate from the neural crest. The resulting hair heterochromia is a lifelong feature, often associated with other pigmentary or sensory anomalies.

Waardenburg Syndrome (WS) is a genetic condition typically characterized by sensorineural hearing loss and pigmentary abnormalities of the hair, skin, and eyes. A white forelock is a common feature in WS, resulting from mutations in genes like PAX3, MITF, or SOX10, which are involved in melanocyte survival and migration.

Another inherited cause is Piebaldism, an autosomal dominant disorder characterized by patches of skin lacking melanocytes (leukoderma) and a white forelock (poliosis). This condition is most commonly linked to a mutation in the KIT proto-oncogene, which interferes with the melanocytes’ ability to migrate from the neural crest. Unlike Waardenburg Syndrome, Piebaldism is usually isolated to the skin and hair, lacking associated hearing loss.

Acquired Factors and Localized Damage

When hair heterochromia develops later in life, it is typically the result of acquired factors, including autoimmune responses, physical trauma, or chemical exposure that damages the melanocytes. These mechanisms disrupt the function of previously healthy pigment cells, leading to localized depigmentation.

Autoimmune conditions represent a major category of acquired causes. In Vitiligo, the immune system mistakenly attacks and destroys the melanocytes in the skin and hair follicles, resulting in distinct white patches of both skin and hair. Alopecia Areata, another immune-mediated disorder, can also lead to hair color changes. The immune reaction in Alopecia Areata may selectively destroy the pigmented hairs, leaving behind only the unpigmented hairs in the affected patch.

Localized physical trauma, such as a severe blow, a burn, or ionizing radiation, can cause permanent damage to a patch of hair follicles. If the injury destroys the melanocyte stem cell reservoir, subsequent hair cycles will produce unpigmented hair. Certain systemic medications or localized chemical treatments can also temporarily or permanently disrupt melanin synthesis, leading to a patch of depigmented hair.