Alopecia areata
Alopecia areata
Alopecia areata, also known as spot baldness, is a
condition in which hair is lost from some or all body areas. It
often results in a few bald spots on the scalp, each about the size of a
coin. Psychological stress and illness are possible
factors in bringing on alopecia areata in individuals at risk, but in most cases,
there is no obvious trigger. People are generally otherwise
healthy. In some instances, all the hair on the scalp is lost (alopecia totalis), or all body hair is lost (alopecia universalis). Hair loss can be permanent,
or temporary.
Alopecia areata is believed to be an autoimmune disease resulting from a breach
in the immune privilege of the hair follicles. Risk factors include a
family history of the condition. Among identical twins, if one is affected, the other has about a 50%
chance of also being affected. The underlying mechanism involves failure
by the body to recognize its own cells, with subsequent immune-mediated
destruction of the hair follicle.
No cure for the condition is known. Some
treatments, particularly triamcinolone injections and 5% minoxidil topical creams, are effective in
speeding hair regrowth. Sunscreen, head coverings to protect from cold and sun,
and Glasses, if the eyelashes are missing, are also
recommended. In more than 50% of cases of sudden-onset localized
"patchy" disease, hair regrows within a year. In patients with only
one or two patches, this one-year recovery will occur in up to
80%. However, many people will have more than one episode throughout a lifetime. In many patients, hair loss and regrowth
occur simultaneously over several years. Among those in
whom all body hair is lost, fewer than 10% recover.
About 0.15% of people are affected at any one time,
and 2% of people are affected at some point in time. Onset is usually in
childhood. Females are affected at higher rates than males.
Signs
and symptoms
The 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 mildly painful. The hair tends to fall
out over a short period, 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. These hairs are very short
(3–4 mm) and can be seen surrounding the bald patches.
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 tends 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. Onychoptosis defluvium, also known as
alopecia unguium, is casting off the nail seen in association with alopecia
areata.
Causes
In alopecia areata, a hair follicle is attacked by the immune system.
T-cells swarm the roots, killing the follicle. This causes the hair to fall out
and parts of the head to become bald.
Alopecia areata is thought to be a systemic
autoimmune disorder in which the body attacks its own anagen hair follicles and suppresses or stops hair growth. For
example, T cell lymphocytes cluster around affected
follicles, causing inflammation and subsequent hair loss. Hair follicles in a
normal state are thought to be kept secure from the immune system, a phenomenon
called immune privilege. A breach in this immune privilege state is considered the cause of alopecia areata. A few cases of babies being born
with congenital alopecia areata have been reported. It
is recognized as a type 1 inflammatory disease.
Alopecia areata is not contagious. It occurs more
frequently in people who have affected family members, suggesting heredity may be a factor. Strong evidence of
genetic association with increased risk for alopecia areata was found by
studying families with two or more affected members. This study identified at
least four regions in the genome that are likely to contain these
genes. In addition, alopecia areata shares genetic risk factors with other
autoimmune diseases, including rheumatoid arthritis, type 1 diabetes, and celiac disease. It may be the only manifestation of celiac
disease.
Endogenous retinAlopecia areata
Alopecia areata, also known as spot baldness, is a
condition in which hair is lost from some or all body areas. It
often results in a few bald spots on the scalp, each about the size of a
coin. Psychological stress and illness are possible
factors in bringing on alopecia areata in individuals at risk, but in most cases,
there is no obvious trigger. People are generally otherwise
healthy. In some instances, all the hair on the scalp is lost (alopecia totalis), or all body hair is lost (alopecia universalis). Hair loss can be permanent,
or temporary.
Alopecia areata is believed to be an autoimmune disease resulting from a breach
in the immune privilege of the hair follicles. Risk factors include a
family history of the condition. Among identical twins, if one is affected, the other has about a 50%
chance of also being affected. The underlying mechanism involves failure
by the body to recognize its own cells, with subsequent immune-mediated
destruction of the hair follicle.
No cure for the condition is known. Some
treatments, particularly triamcinolone injections and 5% minoxidil topical creams, are effective in
speeding hair regrowth. Sunscreen, head coverings to protect from cold and sun,
and glasses, if the eyelashes are missing, are also
recommended. In more than 50% of cases of sudden-onset localized
"patchy" disease, hair regrows within a year. In patients with only
one or two patches, this one-year recovery will occur in up to
80%. However, many people will have more than one episode throughout a lifetime. In many patients, hair loss and regrowth
occur simultaneously over several years. Among those in
whom all body hair is lost, fewer than 10% recover.
About 0.15% of people are affected at any one time,
and 2% of people are affected at some point in time. Onset is usually in
childhood. Females are affected at higher rates than males.
Signs
and symptoms
The 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 mildly painful. The hair tends to fall
out over a short period, 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. These hairs are very short
(3–4 mm), and can be seen surrounding the bald patches.
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 tends 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. Onychoptosis defluvium, also known as
alopecia unguium, is casting off the nail seen in association with alopecia
areata.
Causes
In alopecia areata, a hair follicle is attacked by the immune system.
T-cells swarm the roots, killing the follicle. This causes the hair to fall out
and parts of the head to become bald.
Alopecia areata is thought to be a systemic
autoimmune disorder in which the body attacks its own anagen hair follicles and suppresses or stops hair growth. For
example, T cell lymphocytes cluster around affected
follicles, causing inflammation and subsequent hair loss. Hair follicles in a
normal state are thought to be kept secure from the immune system, a phenomenon
called immune privilege. A breach in this immune privilege state is considered the cause of alopecia areata. A few cases of babies being born
with congenital alopecia areata have been reported. It
is recognized as a type 1 inflammatory disease.
Alopecia areata is not contagious. It occurs more
frequently in people who have affected family members, suggesting heredity may be a factor. Strong evidence of
genetic association with increased risk for alopecia areata was found by
studying families with two or more affected members. This study identified at
least four regions in the genome that are likely to contain these
genes. In addition, alopecia areata shares genetic risk factors with other
autoimmune diseases, including rheumatoid arthritis, type 1 diabetes, and celiac disease. It may be the only manifestation of celiac
disease.
Endogenous retinoid metabolic defect is a
key part of the pathogenesis of the alopecia areata.
In 2010, a genome-wide association study was
completed that identified 129 single nucleotide polymorphisms that were
associated with alopecia areata. The genes that were identified include those
involved in controlling the activation and proliferation of regulatory T cells,
cytotoxic T lymphocyte-associated antigen 4, interleukin-2, interleukin-2
receptor A, and Eos (also known as Ikaros family zinc finger 4), as well as the
human leukocyte antigen. The study also identified two genes, PRDX5 and STX17,
that are expressed in the hair follicle.
A psych-dermatological connection is noted with
impairment in psychiatric comorbidities including mental well-being, self-esteem
and mental disorders acting as pathogenic triggers for alopecia areata.
Diagnosis
Alopecia areata is usually diagnosed based on
clinical features.
Trichoscopy may aid in
establishing the diagnosis. In alopecia areata, trichoscopy shows regularly
distributed "yellow dots" (hyperkeratotic plugs), small
exclamation-mark hairs, and "black dots" (destroyed hairs in the hair
follicle opening).
Oftentimes, however, discrete areas of hair loss
surrounded by exclamation mark hairs are sufficient for the clinical diagnosis of
alopecia areata. Sometimes, reddening of the skin, and erythema, may also be present in the balding area.
A biopsy is rarely needed to make the diagnosis or
aid in the management of alopecia areata. Histologic findings may include
peribulbar lymphocytic infiltration resembling a "swarm of
bees", a shift in the anagen-to-telogen ratio towards telogen, and dilated
follicular infundibulae. Other helpful findings can include pigment
incontinence in the hair bulb and follicular stelae. Occasionally, in
inactive alopecia areata, no inflammatory infiltrates are found.
Classification
Commonly, alopecia areata involves hair loss in one
or more round spots on the scalp.
·
Hair
may also be lost more diffusely over the whole scalp; in which case the
condition is called diffuse alopecia areata.
·
Alopecia
areata monolocularis describes baldness in only one spot. It may occur anywhere
on the head.
·
Alopecia
areata multilocularis refers to multiple areas of hair loss.
·
Ophiasis refers to hair loss in the shape of a wave at
the circumference of the head.
·
The
disease may be limited only to the beard, in which case it is called alopecia
areata barbae.
·
If the
person loses all the hair on the scalp, the disease is then called alopecia areata totalis.
·
If all
body hair, including pubic hair, is lost, the diagnosis then becomes alopecia areata universalis.
Alopecia areata totalis and universalis are rare.
Treatment
The objective assessment of treatment efficacy is
very difficult and spontaneous remission is unpredictable, but if the affected
area is patchy, the hair may regrow spontaneously in many cases. None of
the existing therapeutic options are curative or preventive. A 2020
systematic review showed greater than 50% hair regrowth in 80.9% of patients
treated with 5 mg/mL triamcinolone injections. A Cochrane-style systematic review
published in 2019 showed that 5% topical minoxidil was more than 8x more
associated with >50% hair regrowth at 6 months compared to placebo. In
cases of severe hair loss, limited success has been achieved by using the corticosteroid medications clobetasol or fluocinonide as an injection or
cream. Application of corticosteroid creams to the affected skin is less
effective and takes longer to produce results. Steroid injections are commonly
used in sites where the areas of hair loss on the head are small or especially
where eyebrow hair has been lost. Whether they are effective is uncertain. Some
other medications that have been used are minoxidil, Elocon (mometasone) ointment
(steroid cream), irritants (anthralin or topical coal tar), and topical immunotherapy ciclosporin, sometimes in different combinations. Topical
corticosteroids frequently fail to enter the skin deeply enough to affect the
hair bulbs, which are the treatment target, and small lesions typically
also regrow spontaneously. Oral corticosteroids may decrease hair loss, but
only for the period during which they are taken, and these medications can
cause serious side effects. No one treatment is
effective in all cases, and some individuals may show no response to any
treatment.
For more severe cases, studies have shown promising
results with the individual use of the immunosuppressant methotrexate or
adjunct use with corticosteroids. When alopecia areata is associated with celiac disease, treatment with a gluten-free diet allows for complete and permanent regrowth of the scalp and other body hair in many people, but in others, remissions and
recurrences are seen. This improvement is probably due to the
normalization of the immune response as a result of gluten withdrawal from the diet.
Hair transplantation may be an alternative for
patients with chronic local alopecia areata. The fact that the disease is
autoimmune and progresses with relapses is one of the biggest question marks
before surgery. There have been case reports in the literature since the early
2000s. However, in an article published long-term follow-up; It is reported
that the hair transplanted to the eyebrow area falls out again due to the
recurrence of the disease. A similar situation was not mentioned in
previous studies on this subject. Perhaps the long-term follow-ups of other
studies were not sufficient.
Prognosis
In most cases that begins with a small number of
patches of hair loss, hair grows back after a few months to a year. In
cases with a greater number of patches, hair can either grow back or progress
to alopecia areata totalis or, in rare cases, alopecia areata universalis.
No loss of body function occurs, and the effects of
alopecia areata are psychological (loss of self-image due to hair loss),
although these can be severe. Loss of hair also means the scalp burns more
easily in the sun. Patients may also have aberrant nail formation because keratin forms both hair and nails.
Hair may grow back and then fall out again later.
This may not indicate a recurrence of the condition, but rather a natural cycle
of growth and shedding from a relatively synchronised start; such a pattern
will fade over time. Episodes of alopecia areata before puberty predispose to
chronic recurrence of the condition.
Alopecia can be the cause of psychological stress. Because hair loss can lead to significant changes in appearance, individuals with it may experience social phobia, anxiety, and depression.
A psych-dermatological connection is noted with
impairment in psychiatric comorbidities including mental well-being, self-esteem
and mental disorders acting as pathogenic triggers for alopecia areata.
Diagnosis
Alopecia areata is usually diagnosed based on
clinical features.
Trichoscopy may aid in
establishing the diagnosis. In alopecia areata, trichoscopy shows regularly
distributed "yellow dots" (hyperkeratotic plugs), small
exclamation-mark hairs, and "black dots" (destroyed hairs in the hair
follicle opening).
Oftentimes, however, discrete areas of hair loss surrounded by exclamation mark hairs are sufficient for the clinical diagnosis of alopecia areata. Sometimes, reddening of the skin, and erythema, may also be present in the balding area.
Treatment
The objective assessment of treatment efficacy is
very difficult and spontaneous remission is unpredictable, but if the affected
area is patchy, the hair may regrow spontaneously in many cases. None of
the existing therapeutic options are curative or preventive. A 2020
systematic review showed greater than 50% hair regrowth in 80.9% of patients
treated with 5 mg/mL triamcinolone injections. A Cochrane-style systematic review
published in 2019 showed that 5% topical minoxidil was more than 8x more
associated with >50% hair regrowth at 6 months compared to placebo. In
cases of severe hair loss, limited success has been achieved by using the corticosteroid medications clobetasol or fluocinonide as an injection or
cream. Application of corticosteroid creams to the affected skin is less
effective and takes longer to produce results. Steroid injections are commonly
used in sites where the areas of hair loss on the head are small or especially
where eyebrow hair has been lost. Whether they are effective is uncertain. Some
other medications that have been used are minoxidil, Elocon (mometasone) ointment
(steroid cream), irritants (anthralin or topical coal tar), and topical immunotherapy ciclosporin, sometimes in different combinations. Topical
corticosteroids frequently fail to enter the skin deeply enough to affect the
hair bulbs, which are the treatment target, and small lesions typically
also regrow spontaneously. Oral corticosteroids may decrease hair loss, but
only for the period during which they are taken, and these medications can
cause serious side effects. No one treatment is
effective in all cases, and some individuals may show no response to any
treatment.
For more severe cases, studies have shown promising
results with the individual use of the immunosuppressant methotrexate or
adjunct use with corticosteroids. When alopecia areata is associated with celiac disease, treatment with a gluten-free diet allows for complete and permanent regrowth of the scalp and other body hair in many people, but in others, remissions and
recurrences are seen. This improvement is probably due to the
normalization of the immune response as a result of gluten withdrawal from the diet.
Hair transplantation may be an alternative for
patients with chronic local alopecia areata. The fact that the disease is
autoimmune and progresses with relapses is one of the biggest question marks
before surgery. There have been case reports in the literature since the early
2000s. However, in an article published long-term follow-up; It is reported
that the hair transplanted to the eyebrow area falls out again due to the
recurrence of the disease. A similar situation was not mentioned in
previous studies on this subject. Perhaps the long-term follow-ups of other
studies were not sufficient.
Prognosis
In most cases that begins with a small number of
patches of hair loss, hair grows back after a few months to a year. In
cases with a greater number of patches, hair can either grow back or progress
to alopecia areata totalis or, in rare cases, alopecia areata universalis.
No loss of body function occurs, and the effects of
alopecia areata are psychological (loss of self-image due to hair loss),
although these can be severe. Loss of hair also means the scalp burns more
easily in the sun. Patients may also have aberrant nail formation because keratin forms both hair and nails.
Hair may grow back and then fall out again later.
This may not indicate a recurrence of the condition, but rather a natural cycle
of growth and shedding from a relatively synchronised start; such a pattern
will fade over time. Episodes of alopecia areata before puberty predispose to
chronic recurrence of the condition.
Alopecia can be the cause of psychological stress. Because hair loss can lead to significant changes
in appearance, individuals with it may experience social phobia, anxiety, and depression.
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