Woolly hair is a difficult to brush hair, usually present since birth and typically most severe in childhood. It has extreme curls and

kinks The Kinks were an English rock band formed in Muswell Hill, north London, in 1963 by brothers Ray and Dave Davies. They are regarded as one of the most influential rock bands of the 1960s. The band emerged during the height of British rhythm ...

and occurs in non-black people. The hairs come together to form tight locks, unlike in

afro-textured hair Afro-textured hair, or kinky hair, is a human hair texture originating from sub-Saharan Africa. Each strand of this hair type grows in a tiny, angle-like helix shape. The overall effect is such that, contrasted with straight, wavy, or curly hai ...

, where the hairs remain individual. Woolly hair can be generalised over the whole scalp, when it tends to run in families, or it may involve just part of the scalp as in

woolly hair nevus Woolly hair nevus (alternatively spelled "Wooly hair nevus") is a congenital condition in which hair in a circumscribed area of the scalp is kinked or wool Wool is the textile fibre obtained from sheep and other mammals, especially goats, ...

. The presence of woolly hair may indicate other problems such as with the heart in Naxos–Carvajal syndrome. Diagnosis is suspected by its general appearance and confirmed by scanning electron microscopy. The condition is rare. Alfred Milne Gossage coined the term ''woolly hair'' in 1908.

Edgar Anderson Edgar Shannon Anderson (November 9, 1897 – June 18, 1969) was an American botanist. He introduced the term ''introgressive hybridization'' and his 1949 book of that title was an original and important contribution to botanical genetics. HIs wo ...

distinguished woolly hair from afro-textured hair in 1936.

Discovery

Alfred Milne Gossage coined the term ''woolly hair'' to describe the sign in 18 members in three or four generations of a European family in Lowestoft, England, in 1908. He thought it resembled

, possibly from a Mexican ancestor in that family. He described a dominant inheritance in several members with thick skin of palms and soles, curly hair, and two different coloured eyes, and sent them to

William Bateson William Bateson (8 August 1861 – 8 February 1926) was an English biologist who was the first person to use the term genetics to describe the study of heredity, and the chief populariser of the ideas of Gregor Mendel following their rediscove ...

distinguished woolly hair from Afro-hair in 1936. In 1974 Hutchinson's team classified woolly hair as ''hereditary woolly hair'' (autosomal dominant), ''familial woolly hair'' (autosomal recessive), and ''

''. Woolly hair was found in Naxos syndrome, first described in 1986 in Naxos, Greece, and was noted in

Carvajal syndrome Palmoplantar keratodermas are a heterogeneous group of disorders characterized by abnormal thickening of the stratum corneum of the palms and soles. Autosomal recessive, dominant, X-linked, and acquired forms have all been described. Types C ...

, first described in 1998, in Ecuador.

Cause

Woolly hair may run in families and either occur on its own, or as part of a syndrome.

Hereditary woolly hair

Hereditary woolly hair is autosomal dominant.

Familial woolly hair

Familial woolly hair is autosomal recessive. It may be part of a syndrome such as Naxos syndrome, due to passing on of mutations in the '' JUP gene''. When part of Carvajal syndrome, it is due the passing of mutations of the '' Desmoplakin gene''. The two syndromes caused by two different genes, are considered as one entity; Naxos–Carvajal syndrome.

Woolly hair nevus

The woolly hair of a woolly hair nevus is in a circumscribed area of the scalp, appears in infancy and does not run in families. It likely represents a

mosaic A mosaic is a pattern or image made of small regular or irregular pieces of colored stone, glass or ceramic, held in place by plaster/mortar, and covering a surface. Mosaics are often used as floor and wall decoration, and were particularly pop ...

RASopathy The RASopathies are developmental syndromes caused by germline mutations (or in rare cases by somatic mosaicism) in genes that alter the Ras subfamily and mitogen-activated protein kinases that control signal transduction, including: * Capill ...

Signs and symptoms

Woolly hair is typically very curly, kinky and characteristically impossible to brush. It can be generalised over the whole scalp, or involve just part of the scalp, and occurs in non-black people. The hairs come together to form tight locks, whereas in

the hairs remain individual. The hairs typically remain shorter than and may be slightly

lighter A lighter is a portable device which creates a flame, and can be used to ignite a variety of items, such as cigarettes, gas lighter, fireworks, candles or campfires. It consists of a metal or plastic container filled with a flammable liquid or ...

in colour. Woolly hair nevus is a localised area of woolly hair, which may occur on its own, or appear as dark twisted and kinking hair in an adult. Half of people with woolly hair nevus have a warty skin lesion on the same side of the body. It may be associated with eye problems such as two different coloured eyes or strands of tissue across the pupil of the eye. Other associations include ear problems, kidney disease, tooth decay, impairment of bone growth, and skin lesions. Generalised woolly hair is typically seen in Naxos–Carvajal syndrome (with heart involvement),

Noonan syndrome Noonan syndrome (NS) is a genetic disorder that may present with mildly unusual facial features, short height, congenital heart disease, bleeding problems, and skeletal malformations. Facial features include widely spaced eyes, light-colored ...

, and

cardiofaciocutaneous syndrome Cardiofaciocutaneous (CFC) syndrome is an extremely rare genetic disorder, and is one of the RASopathies. It was first described in 1986. It is characterized by the following: *Distinctive facial appearance *Unusually sparse, brittle, curly scal ...

Diagnosis

Diagnosis is suspected by its general appearance and confirmed by scanning electron microscopy. Microscopy,

trichoscopy Trichoscopy is a method of hair and scalp evaluation and is used for diagnosing hair and scalp diseases. The method is based on dermoscopy. In trichoscopy hair and scalp structures may be visualized at many-fold magnification. Currently magnificat ...

and

dermoscopy Dermatoscopy also known as dermoscopy or epiluminescence microscopy, is the examination of skin lesions with a dermatoscope. It is a tool similar to a camera to allow for inspection of skin lesions unobstructed by skin surface reflections. The de ...

also play a role. The hair strand typically has a smaller diameter, is ovoid on cross-section and exhibits abnormal twisting. The hair shaft also has weak points and alternating dark and light bands. The hair shaft is characteristically of a "snake crawl appearance". Dermoscopy may be required to recognise skin signs.

Discovery

Cause

Hereditary woolly hair

Familial woolly hair

Woolly hair nevus

Signs and symptoms

Diagnosis

Outcome

Epidemiology

See also

References

Further reading