Alopecia areata

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Alopecia areata
Other namesAlopecia Celsi, vitiligo capitis, Jonston's alopecia [1]
Allopecia areata.JPG
Alopecia areata seen on the back of the scalp
Pronunciation
Specialty Dermatology Immunology [6]
Symptoms Areas of hair loss, usually on the scalp [7]
Usual onsetChildhood [7]
Causes Autoimmune [7]
Risk factors Family history, female sex, rheumatoid arthritis, type 1 diabetes, celiac disease [7] [8] [9]
Differential diagnosis Trichotillomania, alopecia mucinosa, postpartum alopecia [1]
Treatment Sunscreen, head coverings to protect from sun and cold [7]
Medication topical minoxidil [10] and triamcinolone injections [11]
Prognosis Does not affect life expectancy [7] [1]
Frequency~2% (US) [7]

Alopecia areata, also known as spot baldness, is a condition in which hair is lost from some or all areas of the body. [12] [1] It often results in a few bald spots on the scalp, each about the size of a coin. [7] 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. [7] People are generally otherwise healthy. [7] In a few cases, 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. [7] [1]

Contents

Alopecia areata is believed to be an autoimmune disease resulting from a breach in the immune privilege of the hair follicles. [12] [13] Risk factors include a family history of the condition. [7] Among identical twins, if one is affected, the other has about a 50% chance of also being affected. [7] The underlying mechanism involves failure by the body to recognize its own cells, with subsequent immune-mediated destruction of the hair follicle. [7]

No cure for the condition is known. [7] Some treatments, particularly triamcinolone injections and 5% minoxidil topical creams, [11] [10] are effective in speeding hair regrowth. [7] [1] Sunscreen, head coverings to protect from cold and sun, and glasses, if the eyelashes are missing, are also recommended. [7] In more than 50% of cases of sudden-onset localized "patchy" disease, hair regrows within a year. [14] [15] [7] In patients with only one or two patches, this one-year recovery will occur in up to 80%. [16] [17] However, many people will have more than one episode over the course of a lifetime. [15] In many patients, hair loss and regrowth occurs simultaneously over the course of several years. [7] Among those in whom all body hair is lost, fewer than 10% recover. [18]

About 0.15% of people are affected at any one time, and 2% of people are affected at some point in time. [7] [18] Onset is usually in childhood. [7] Females are affected at higher rates than males. [9]

Signs and symptoms

Alopecia areata Alopecia areata.jpg
Alopecia areata

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. [19] Alopecia areata most often affects the scalp and beard, but may occur on any part of the body with hair. [20] 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.[ citation needed ]

The area of hair loss may tingle or be mildly painful. [21] 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. [22]

Exclamation point hairs, narrower along the length of the strand closer to the base, producing a characteristic "exclamation point" appearance, are often present. [22] These hairs are very short (3–4 mm), and can be seen surrounding the bald patches. [17]

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. [23]

Nails may have pitting or trachyonychia. [20] Onychoptosis defluvium, also known as alopecia unguium, is casting off the nail seen in association with alopecia areata. [24] : 660 

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.svg
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. [22] 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 as the cause of alopecia areata. [13] A few cases of babies being born with congenital alopecia areata have been reported. [25] It is recognized as a type 1 inflammatory disease. [26]

Alopecia areata is not contagious. [22] It occurs more frequently in people who have affected family members, suggesting heredity may be a factor. [22] 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. [27] In addition, alopecia areata shares genetic risk factors with other autoimmune diseases, including rheumatoid arthritis, type 1 diabetes, and celiac disease. [8] It may be the only manifestation of celiac disease. [28] [29]

Endogenous retinoids metabolic defect is a key part of the pathogenesis of the alopecia areata. [30]

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. [31]

A psychodermatological connection is noted with impairment in psychiatric comorbidities including mental well-being, self esteem and mental disorders acting as pathogenic triggers for alopecia areata. [32] [33] [34] [35]

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). [36]

Oftentimes, however, discrete areas of hair loss surrounded by exclamation mark hairs is sufficient for clinical diagnosis of alopecia areata. Sometimes, reddening of the skin, erythema, may also be present in the balding area. [18]

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. [8] Other helpful findings can include pigment incontinence in the hair bulb and follicular stelae. Occasionally, in inactive alopecia areata, no inflammatory infiltrates are found.[ citation needed ]

Classification

Varying degrees of alopecia areata, along with alopecia totalis Fox Plate V.jpg
Varying degrees of alopecia areata, along with alopecia totalis

Commonly, alopecia areata involves hair loss in one or more round spots on the scalp. [22] [37]

Alopecia areata totalis and universalis are rare. [38]

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. [39] None of the existing therapeutic options are curative or preventive. [39] A 2020 systematic review showed greater than 50% hair regrowth in 80.9% of patients treated with 5 mg/mL triamcinolone injections. [11] A Cochrane-style systematic review published in 2019 showed 5% topical minoxidil was more than 8x more associated with >50% hair regrowth at 6 months compared to placebo. [10] 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.[ citation needed ] 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, [20] and small lesions typically also regrow spontaneously. Oral corticosteroids may decrease the hair loss, but only for the period during which they are taken, and these medications can cause serious side effects. [20] No one treatment is effective in all cases, and some individuals may show no response to any treatment. [40]

For more severe cases, studies have shown promising results with the individual use of the immunosuppressant methotrexate or adjunct use with corticosteroids. [41]

When alopecia areata is associated with celiac disease, treatment with a gluten-free diet allows for complete and permanent regrowth of scalp and other body hair in many people, but in others, remissions and recurrences are seen. [28] This improvement is probably due to the normalization of the immune response as a result of gluten withdrawal from the diet. [28]

In June 2022, the U.S. Food and Drug Administration (FDA) authorized baricitinib, a Janus kinase (JAK) inhibitor, for the treatment of severe alopecia areata. [42]

Ritlecitinib (Litfulo) was approved for medical use in the United States in June 2023. [43]

Fecal matter transplants (FMT) have been shown to reverse AA and support hair growth, with long lasting results, even going as far as growing additional hair on arms and face while grey hairs even regained colour. This supports the idea of a connection between gut microbiota having a part in hair loss. [44]

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. [45] A similar situation was not mentioned in previous studies on this subject. Perhaps the long-term follow-ups of other studies were not sufficient.

Deuruxolitinib (Leqselvi) was approved for medical use in the United States in July 2024. [46]

Prognosis

In most cases that begin with a small number of patches of hair loss, hair grows back after a few months to a year. [21] 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. [21]

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.[ citation needed ]

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. [20]

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. [47]

Epidemiology

The condition affects 0.1%–0.2% of the population, with a lifetime risk of 1%-2%, [48] and is more common in females. [9]

Alopecia areata occurs in people who are otherwise healthy and have no other skin disorders. [20] Initial presentation most commonly occurs in the early childhood, late teenage years, or young adulthood, but can happen at any ages. [22] Patients also tend to have a slightly higher incidence of conditions related to the immune system, such as asthma, allergies, atopic dermatitis, and hypothyroidism.

Society and culture

The term alopecia, used by physicians dating back to Hippocrates, originates from the Greek word for fox, "alopex", and was so-named due to fur loss seen in fox mange. "Areata" is derived from the Latin word, "area", meaning a vacant space or patch. [49]

Alopecia areata and alopecia barbae have been identified by some as the biblical nethek condition that is part of the greater tzaraath family of skin disorders; the said disorders are purported to being discussed in the Book of Leviticus, chapter 13. [50]

Notable people

NASCAR driver Joey Logano, obstacle athlete Kevin Bull, [51] politicians Peter Dutton [52] and Ayanna Pressley, [53] K-pop singer Peniel Shin of BtoB, [54] actors Christopher Reeve, [55] Anthony Carrigan [56] and Alan Fletcher, [57] and actresses Jada Pinkett Smith, [58] May Calamawy, [59] and Lili Reinhart [60] all have some form of alopecia areata.

Research

Many medications are being studied. [61]

In 2014, preliminary findings showing that oral ruxolitinib, a drug approved by the US Food and Drug Administration (FDA) for bone marrow disorder myelofibrosis, restored hair growth in three individuals with long-standing and severe disease. [62]

In March 2020, the US FDA granted breakthrough therapy designation to baricitinib for the systematic treatment of alopecia areata [63] and granted approval in June 2022, [42] with a 32% efficacy rate for people with 50% hair loss reaching 80% scalp coverage in 36 weeks. [64] It acts as an inhibitor of janus kinase (JAK), blocking the subtypes JAK1 and JAK2. [65]

Related Research Articles

<span class="mw-page-title-main">Hair loss</span> Loss of hair from the head or body

Hair loss, also known as alopecia or baldness, refers to a loss of hair from part of the head or body. Typically at least the head is involved. The severity of hair loss can vary from a small area to the entire body. Inflammation or scarring is not usually present. Hair loss in some people causes psychological distress.

<span class="mw-page-title-main">Hair follicle</span> Organ found in mammalian skin

The hair follicle is an organ found in mammalian skin. It resides in the dermal layer of the skin and is made up of 20 different cell types, each with distinct functions. The hair follicle regulates hair growth via a complex interaction between hormones, neuropeptides, and immune cells. This complex interaction induces the hair follicle to produce different types of hair as seen on different parts of the body. For example, terminal hairs grow on the scalp and lanugo hairs are seen covering the bodies of fetuses in the uterus and in some newborn babies. The process of hair growth occurs in distinct sequential stages: anagen is the active growth phase, catagen is the regression of the hair follicle phase, telogen is the resting stage, exogen is the active shedding of hair phase and kenogen is the phase between the empty hair follicle and the growth of new hair.

<span class="mw-page-title-main">Telogen effluvium</span> Medical condition

Telogen effluvium is a scalp disorder characterized by the thinning or shedding of hair resulting from the early entry of hair in the telogen phase. It is in this phase that telogen hairs begin to shed at an increased rate, where normally the approximate rate of hair loss is 125 hairs per day.

<span class="mw-page-title-main">Alopecia universalis</span> Medical condition

Alopecia universalis(AU), also known as alopecia areata universalis, is a medical condition involving the loss of all body hair, including eyebrows, eyelashes, chest hair, armpit hair, and pubic hair. It is the most severe form of alopecia areata (AA). People with the condition are usually healthy and have no other symptoms and a normal life expectancy.

The management of hair loss, includes prevention and treatment of alopecia, baldness, and hair thinning, and regrowth of hair.

<span class="mw-page-title-main">Alopecia totalis</span> Medical condition

Alopecia totalis is the loss of all hair on the head and face. Its causes are unclear, but believed to be autoimmune. Research suggests there may be a genetic component linked to developing alopecia totalis; the presence of DRB1*0401 and DQB1*0301, both of which are human leukocyte antigens (HLA), were found to be associated with long-standing alopecia totalis.

<span class="mw-page-title-main">Pattern hair loss</span> Medical condition

Pattern hair loss is a hair loss condition that primarily affects the top and front of the scalp. In male-pattern hair loss (MPHL), the hair loss typically presents itself as either a receding front hairline, loss of hair on the crown and vertex of the scalp, or a combination of both. Female-pattern hair loss (FPHL) typically presents as a diffuse thinning of the hair across the entire scalp.

Ophiasis is a form of alopecia areata characterized by the loss of hair in the shape of a wave at the circumference of the head.

<span class="mw-page-title-main">Poliosis</span> Medical condition

Poliosis circumscripta, commonly referred to as a "white forelock," is a condition characterized by localized patches of white hair due to a reduction or absence of melanin in hair follicles. Although traditionally associated with the scalp, poliosis can affect any hairy area on the body, including eyebrows, eyelashes, and beards. Microscopically, poliosis is marked by the lack of melanin or melanocytes in the hair bulbs, though epidermal melanocytes typically remain unaffected unless associated with conditions like vitiligo.

<span class="mw-page-title-main">Loose anagen syndrome</span> Medical condition

Loose anagen syndrome, also known as loose anagen hair syndrome, is a hair disorder related to dermatology. It is characterised by the easy and pain free detachment of anagen staged hairs from the scalp. This hair condition can be spontaneous or genetically inherited.

Scarring hair loss, also known as cicatricial alopecia, is the loss of hair which is accompanied with scarring. This is in contrast to non scarring hair loss.

Pressure alopecia, also known as postoperative alopecia, and pressure-induced alopecia, occurs in adults after prolonged pressure on the scalp during general anesthesia, with the head fixed in one position, and may also occur in chronically ill persons after prolonged bed rest in one position that causes persistent pressure on one part of the scalp, all likely due to pressure-induced ischemia.

Non scarring hair loss, also known as noncicatricial alopecia is the loss of hair without any scarring being present. There is typically little inflammation and irritation, but hair loss is significant. This is in contrast to scarring hair loss during which hair follicles are replaced with scar tissue as a result of inflammation. Hair loss may be spread throughout the scalp (diffuse) or at certain spots (focal). The loss may be sudden or gradual with accompanying stress.

Alopecia mucinosa, also known as Follicular mucinosis, Mucinosis follicularis, Pinkus' follicular mucinosis, and Pinkus' follicular mucinosis–benign primary form, is a skin disorder that generally presents, but not exclusively, as erythematous plaques or flat patches without hair primarily on the scalp, neck and face. This can also be present on the body as a follicular mucinosis and may represent a systemic disease.

<span class="mw-page-title-main">Trichoscopy</span>

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 magnifications ranging from 10-fold to 70-fold are most popular in research and clinical practice.

Antonella Tosti is an Italian physician and scientist with major contributions in the field of dermatology, including developing dermoscopy for the diagnosis and care of hair diseases, a world recognized expert in hair disorders. Her contributions to knowledge about nails include research about videodermoscopy of the hyponychium and the nail plate.

<span class="mw-page-title-main">Frontal fibrosing alopecia</span> Medical condition

Frontal fibrosing alopecia is the frontotemporal hairline recession and eyebrow loss in postmenopausal women that is associated with perifollicular erythema, especially along the hairline. It is considered to be a clinical variant of lichen planopilaris.

The growth of human hair occurs everywhere on the body except for the soles of the feet, the palms of the hands, the inside of the mouth, the lips, the backs of the ears, some external genital areas, the navel, and, apart from eyelashes, the eyelids. Hair is a stratified squamous keratinized epithelium made of multi-layered flat cells whose rope-like filaments provide structure and strength to the hair shaft. The protein called keratin makes up hair and stimulates hair growth. Hair follows a specific growth cycle with three distinct and concurrent phases: anagen, catagen, and telogen. Each phase has specific characteristics that determine the length of the hair.

Lipedematous alopecia is a disorder characterized by a thick boggy scalp and hair loss. Symptoms include pain, puritis, headaches, and paresthesia. The cause of lipedematous alopecia is unknown. The diagnosis is made my excluding other disorders, MRI, CT scan, and histopathology. There is no accepted treatment however surgical debunking has been found effective.

Frictional alopecia is the loss of hair that is caused by rubbing of the hair, follicles, or skin around the follicle. The most typical example of this is the loss of ankle hair among people who wear socks constantly for years. The hair may not grow back even years after the source of friction has ended.

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