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Among the symptoms(and signs) for this condition are the following:
- lack of apparent hair growth
- hair appears patchy
- hair breaks easily close to scalp
- hair may have thickenings or nodes in the shaft
- ends of hair thinned or split
- whitish discoloration of hair tips
- hair breaks easily at tips
The presentation may be of alopecia (baldness). Individuals vary in severity of symptoms. Nail deformities may also be present as well as hair follicle keratosis and follicular hyperkeratosis.
In humans, hair is commonly present on all the basal segments of the digits and invariably absent from all the terminal ones. On the middle segments, there is wide fluctuation with apparent familial and racial tendencies. Hair is present on the middle segment of the fingers more frequently than on the middle segment of the toes. Hair is most often found on the middle segment of the fourth finger.
CCCA usually begins at the central (sagittal) midline of the scalp. It is symmetric and exhibits scarring as the name suggests. It involves solely the top of the scalp or may progress to Hamilton–Norwood scale Type VI or VII. Early symptoms may include pruritus, dysesthesias and tenderness. On examination the skin is thin with few follicular ostia and later in the disease the scalp may appear shiny.
Hair diseases are disorders primarily associated with the follicles of the hair.
A few examples are
- Alopecia
- Bubble hair deformity
- Hair casts
- Hair loss
- hypertrichosis
- Ingrown hair
- Monilethrix
- Premature greying of hair
- Pattern hair loss
- Trichorrhexis invaginata
Many hair diseases can be associated with distinct underlying disorders.
Piedra are fungal diseases.
Hair disease may refer to excessive shedding or baldness (or both). Balding can be localised or diffuse, scarring or non-scarring. Increased hair can be due to hormonal factors (hirsutism) or non-hormonal (hypertrichosis). Scalp disorders may or may not be associated with hair loss.
Trichorrhexis nodosa is a defect in the hair shaft characterized by thickening or weak points (nodes) that cause the hair to break off easily. This group of conditions contributes to the appearance of hair loss, lack of growth, and damaged-looking hair.
Midphalangeal hair, or the presence/absence of hair on the middle phalanx of the ring finger, is one of the most widely studied markers in classical genetics of human populations. Although this polymorphism was observed at other fingers as well, for this kind of research, the fourth finger of the hand has been conventionally selected.
The hair is normal in quantity and is usually silvery-blond or straw-colored. It is disorderly, it stands out from the scalp, and cannot be combed flat. The underlying structural anomaly is longitudinal grooving of the hair shaft, which appears triangular in cross section. This is caused by mutations in one of three possible genes; "PADI3", "TGM" or "TCHH3." The characteristic hair shaft anomaly can be demonstrated in asymptomatic family members by scanning electron microscopy. To be noticeable, 50% of hairs must be affected by the structural abnormality. Improvement often occurs in later childhood. An autosomal dominant mode of inheritance has been suggested though an autosomal recessive pattern with varying degrees of penetrance has also been noted. The stiffness of the uncombable hair has been reasoned to be due to the triangular form of the hair shaft in cross section. It has been suggested that the condition may result from premature keratinization of the inner root sheath.
Uncombable hair syndrome, also known as Pili trianguli et canaliculi, Spun-glass hair, and Cheveux incoiffables, is a rare structural anomaly of the hair with a variable degree of effect. It was first reported in the early 20th century and was described in the 1970s. It becomes apparent from as little as 3 months to up to 12 years of age.
Auricular hypertrichosis ("hypertrichosis lanuginosa acquisita", "hypertrichosis pinnae auris") is a genetic condition expressed as long and strong hairs growing from the helix of the pinna.
Trichorrhexis invaginata (also known as "Bamboo hair" ) is a distinctive hair shaft abnormality that may occur sporadically, either in normal hair or with other hair shaft abnormalities, or regularly as a marker for Netherton's syndrome. The primary defect appears to be abnormal keratinization of the hair shaft in the keratogenous zone, allowing for intussusception of the fully keratinized and hard distal shaft into the incompletely keratinized and soft proximal portion of the shaft.
Monilethrix (also referred to as beaded hair) is a rare autosomal dominant hair disease that results in short, fragile, broken hair that appears beaded. It comes from the Latin word for necklace ("monile") and the Greek word for hair ("thrix").
In 1937, Touraine and Solente first noted the association between hair-shaft defects (bamboo node) and ichthyosiform erythroderma. Còme first coined the term ichthyosis linearis circumflexa in 1949, although Rille had previously recorded the distinctive features of ichthyosis linearis circumflexa by 1922. In 1958, Netherton described a young girl with generalized scaly dermatitis and fragile nodular hair-shaft deformities, which he termed trichorrhexis nodosa. Later, this was more appropriately renamed as trichorrhexis invaginata (bamboo hair) for a ball-and-socket–type hair-shaft deformity at the suggestion of Wilkinson et al.
In 1974, Mevorah et al. established the clinical relationship between ichthyosis linearis circumflexa and Netherton syndrome, and an atopic diathesis was found to occur in approximately 75% of patients with Netherton syndrome.
Ear hair generally refers to the terminal hair arising from follicles inside the external auditory meatus in humans. In its broader sense, "ear hair" may also include the fine vellus hair covering much of the ear, particularly at the prominent parts of the anterior ear, or even the abnormal hair growth as seen in hypertrichosis and hirsutism. Medical research on the function of ear hair is currently very scarce.
Hair growth within the ear canal is often observed to increase in older men, together with increased growth of nose hair. Visible hair that protrudes from the ear canal is sometimes trimmed for cosmetic reasons. Excessive hair growth within or on the ear is known medically as "auricular hypertrichosis". Some men, particularly in the male population of India, have coarse hair growth along the lower portion of the helix, a condition referred to as "having "hairy pinnae"" ("hypertrichosis lanuginosa acquisita").
Traction alopecia is a form of alopecia, or gradual hair loss, caused primarily by pulling force being applied to the hair. This commonly results from the sufferer frequently wearing their hair in a particularly tight ponytail, pigtails, or braids. It is also seen occasionally in long-haired people who use barrettes to keep hair out of their faces. Traction alopecia is recession of the hairline due to chronic traction, or hair pulling, and is characterized by a fringe along the marginal hairline on physical exam. Even though this "fringe sign" is considered a useful clinical marker of this condition, cases of frontal fibrosing alopecia presenting with an unusual retention of the hairline (pseudo-fringe sign) have been described.
Symptoms of hair loss include hair loss in patches usually in circular patterns, dandruff, skin lesions, and scarring. Alopecia areata (mild – medium level) usually shows in unusual hair loss areas e.g. eyebrows, backside of the head or above the ears where usually the male pattern baldness does not affect. In male-pattern hair loss, loss and thinning begin at the temples and the crown and either thins out or falls out. Female-pattern hair loss occurs at the frontal and parietal.
People have between 100,000 and 150,000 hairs on their head. The number of strands normally lost in a day varies, but on average is 100. In order to maintain a normal volume, hair must be replaced at the same rate at which it is lost. The first signs of hair thinning that people will often notice are more hairs than usual left in the hairbrush after brushing or in the basin after shampooing. Styling can also reveal areas of thinning, such as a wider parting or a thinning crown.
Bubble hair deformity is an abnormality of the hair shaft.
It is characterized by rows of bubbles seen microscopically within localized areas of brittle hair.
Hidrotic ectodermal dysplasia 2, or Clouston syndrome (referred to as HED2 throughout this entry) is characterized by partial or total alopecia, dystrophy of the nails, hyperpigmentation of the skin (especially over the joints), and clubbing of the fingers. Sparse scalp hair and dysplastic nails are seen early in life. In infancy, scalp hair is wiry, brittle, patchy, and pale; progressive hair loss may lead to total alopecia by puberty. The nails may be milky white in early childhood; they gradually become dystrophic, thick, and distally separated from the nail bed. Palmoplantar keratoderma may develop during childhood and increases in severity with age. The clinical manifestations are highly variable even within the same family.
Central centrifugal cicatricial alopecia (CCCA), also referred to as hot comb alopecia and follicular degeneration syndrome, is a type of alopecia first noticed in African Americans in the 1950s and reported by LoPresti et al. in 1968 as a result of application of petrolatum followed by a stove-heated iron comb. The original theory was that the hot petrolatum would travel down to the hair root, burn the follicle, and after repetitive injury scarring would result. Later CCCA was realized to affect men and women without a history significant for use of such styling techniques. Consequently, the terms "follicular degeneration syndrome" per Sperling and Sau in 1992 and then CCCA per Olsent et al. in 2003 were evolved. Plausible contributing factors may include other African-American styling techniques such as relaxers, tight braids, heavy extensions, certain oils, gels or pomades.
Hypotrichosis ("" + "" + "") is a condition of abnormal hair patterns, predominantly loss or reduction. It occurs, most frequently, by the growth of vellus hair in areas of the body that normally produce terminal hair. Typically, the individual's hair growth is normal after birth, but shortly thereafter the hair is shed and replaced with sparse, abnormal hair growth. The new hair is typically fine, short and brittle, and may lack pigmentation. Baldness may be present by the time the subject is 25 years old.
Hypotrichosis is a common feature of Hallermann–Streiff syndrome as well as others. It can also be used to describe the lack of hair growth due to chemotherapy.
The opposite of hypotrichosis is hypertrichosis, where terminal hair (thick) grows in areas that would otherwise normally have vellus hair (thin), for example abnormally thick facial hair growth in women.
Baldness is the partial or complete lack of hair growth, and part of the wider topic of "hair thinning". The degree and pattern of baldness varies, but its most common cause is androgenic hair loss, "alopecia androgenetica", or "alopecia seborrheica", with the last term primarily used in Europe.
Congenital forms of hypertrichosis are caused by genetic mutations, and are extremely rare, unlike acquired forms. Congenital hypertrichosis is always present at birth.
- Hypertrichosis lanuginosa
Congenital hypertrichosis lanuginosa is noticeable at birth, with the infant completely covered in thin lanugo hair. In normal circumstances, lanugo hair is shed before birth and replaced by vellus hair; however, in a person with congenital hypertrichosis lanuginosa, the lanugo hair remains after birth. The palms of the hands, soles of the feet, and mucous membranes are not affected. As the person ages, the lanugo hair may thin, leaving only limited areas of hypertrichosis.
- Generalized hypertrichosis
Congenital generalized hypertrichosis causes males to exhibit excessive facial and upper body hair, whereas women exhibit less severe asymmetrical hair distribution. The palms, soles, and mucous membranes are not affected.
- Terminal hypertrichosis
Congenital terminal hypertrichosis is characterized by the presence of fully pigmented terminal hair that covers the entire body. This condition is usually accompanied by gingival hyperplasia. This form is most responsible for the term "werewolf syndrome" because of the thick, dark hair that appears. People with this condition are sometimes performers at circuses because of their unusual appearance.
- Circumscribed hypertrichosis
Congenital circumscribed hypertrichosis is associated with the presence of thick vellus hair on the upper extremities. Circumscribed signifies this type of hypertrichosis is restricted to certain parts of the body, in this case, the extensor surfaces of the upper extremities. Hairy elbow syndrome, a type of congenital circumscribed hypertrichosis, shows excessive growth on and around the elbows. This type of hypertrichosis is present at birth, becomes more prominent during aging, and regresses at puberty.
- Localized hypertrichosis
Congenital localized hypertrichosis is a localized increase in hair density and length.
- Nevoid hypertrichosis
Nevoid hypertrichosis may be present at birth or appear later in life. It features an isolated area of excessive terminal hair and is usually not related to any other diseases.
Typical first symptoms of alopecia areata are small bald patches. The underlying skin is unscarred and looks superficially normal. Although these patches can take many shapes, they are usually round or oval. Alopecia areata most often affects the scalp and beard, but may occur on any part of the body with hair. Different areas of the skin may exhibit hair loss and regrowth at the same time. The disease may also go into remission for a time, or may be permanent. It is common in children.
The area of hair loss may tingle or be painful. The hair tends to fall out over a short period of time, with the loss commonly occurring more on one side of the scalp than the other.
Exclamation point hairs, narrower along the length of the strand closer to the base, producing a characteristic "exclamation point" appearance, are often present.
When healthy hair is pulled out, at most a few should come out, and ripped hair should not be distributed evenly across the tugged portion of the scalp. In cases of alopecia areata, hair will tend to pull out more easily along the edge of the patch where the follicles are already being attacked by the body's immune system than away from the patch where they are still healthy.
Nails may have pitting or trachyonychia.
It is commonly seen with certain hair styles or braiding patterns that pull the hairline forcefully towards the vertex of the scalp, and has been reported more often in African American women (as some wear their hair tightly pulled back), in whom it can cause scarring. It has also been seen in female ballerinas, and in cultural traditions where the hair is voluntarily not cut in religious obeisance, the latter caused by progressively increasing weight of the hair itself. Traction alopecia is mechanical in cause, rather than androgenic, and treatment is typically not pharmaceutical. Management includes cessation of the chronic traction, cosmeses, with surgical restoration reserved for more severe cases.
Traction alopecia is a substantial risk in hair weaves, which can be worn either to conceal hair loss, or purely for cosmetic purposes. The former involves creating a braid around the head below the existing hairline, to which an extended-wear hairpiece, or wig, is attached. Since the hair of the braid is still growing, it requires frequent maintenance, which involves the hairpiece being removed, the natural hair braided again, and the piece snugly reattached. The tight braiding and snug hairpiece cause tension on the hair that is already at risk for falling out. Traction Alopecia is one of the most common causes of hair loss in African American women.
Sikh men are also susceptible to traction alopecia if the hair under the turban is tied too tightly for many years.
Other causes include:
- Hairstyle. Although the aforementioned style is one of the culprits, hairstyles such as dreadlocks and single (extension) braids can also have the same effect. Men and women who have suffered from traction alopecia have found that the hair loss occurs most at the hair line—primarily around the temples and the sides of their heads.
- Headgear. Compressive safety helmets worn tightly and closely to the scalp are a cause of traction alopecia. The lining of tightly fitted safety helmets like those worn for activities such as motorcycling, cycling, skiing and snowboarding are responsible for the constant rubbing and tugging of localised areas of the hair and scalp. Frequent wearers or those who use such helmets for prolonged periods seem more likely to suffer traction alopecia.
- Chemicals. A condition known as CCCA (central cicatricial centrifugal alopecia), seen almost exclusively in African American women, can cause extensive hair loss. It is caused by a combination of too much stress (traction) on the hair and the use of harsh relaxers and dyes.
Important diagnoses to consider include female pattern hair loss (FPHL), chronic telogen effluvium (CTE), and alopecia areata (AA). FPHL is a non-scarring progressive miniaturization of the hair follicle with one of three different characteristic patterns. CTE is an idiopathic disease causing increased hair shedding and bi-temporal recession, usually in middle aged women. AA is an autoimmune attack of hair follicles that usually causes hair to fall out in small round patches.