THE HAIR LOSS DIRECTORY
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The History of Alopecia Areata
A definition of Alopecia Areata Alopecia Areata (AA) is a non-scarring, inflammatory, hair loss disease that can affect men, women and children. The factors that activate the onset of Alopecia Areata and the mechanisms of its development are not fully understood. Circumstantial evidence suggests Alopecia Areata is an autoimmune disease where cells of an individual's own immune system prevent hair follicles from producing hair fiber. Although Alopecia Areata is not life threatening, the pressures of an image orientated society can make hair loss psychologically devastating for those affected, their families, and friends.
A brief history of Alopecia Areata
Although Hippocrates first used the term Alopecia (literally translated as "fox's disease"), the characteristics of the hair loss disease we now know to be Alopecia Areata (AA) were first described by Cornelius Celsus in 30 A.D. Celsus described two forms of Alopecia. The first he described as complete baldness occurring in people of all ages. The second he called "ophiasis", which literally translates as "snake", due to the winding way the bald region spread across the skin. He suggested ophiasis was only seen in children. Alopecia Areata is sometimes known as "area celsi" in tribute to Cornelius Celsus. Alopecia Areata has been given many different names throughout history. However, the actual term "Alopecia Areata" was first used by Sauvages in his "Nosologica Medica", published in 1760 in Lyons, France.
From the 1800's onwards there was considerable debate about the cause of Alopecia Areata. Two main hypotheses were put forward, one based on parasitic infection (Gruby 1843, Radcliffe-Crocker 1903), the other based on a nervous disorder (Von Barensrung 1858). The parasitic hypothesis drew support from the way the hair loss lesion developed - slowly expanding in size just as a local infection would. Even more significant were the apparent epidemics of Alopecia Areata reported to occur in institutions such as orphanages and schools (Bowen 1899, Colcott Fox 1913, Davis 1914). However, many attempts to isolate an infective organism and to transfer Alopecia Areata by inoculation have failed (Sabouraud 1896, Ormsby 1948, Ikeda 1967).
The initiation of Alopecia Areata by a nervous disorder, variously known as the trophoneurotic, neurotrophic, or neuropathic hypothesis, eventually gained the support of most dermatologists of the day. This vague hypothesis could be supported by the apparently frequent clinical observations of emotional or physical stress and trauma that were associated with the onset of Alopecia Areata and often reported in the medical journals at that time (Sequeira 1913, Kingsbury 1909). Emotional stress and physical damage were believed to adversely affect hair follicles via the nervous system and Joseph (1886) showed that patchy hair loss could apparently be induced by cutting nerves in the necks of cats (it was later suggested that the hair loss was actually due to the cats scratching themselves). This highly circumstantial, but compelling evidence drew popular support for the trophoneurotic hypothesis. The idea circulated among dermatologists for many years because it was (and still is) very difficult to fundamentally prove or disprove that Alopecia Areata was a nervous disorder. The hypothesis is still supported by some dermatologists today.
One of the more unusual variations on the neuropathic origin of Alopecia Areata was put forward by Jacquet (1902) who suggested Alopecia Areata was initiated by sources of nerve irritation such as defective and diseased teeth. Jacquet's hypothesis was apparently confirmed by others (Decelle 1909). Although Baily (1910) showed dental disease to be equally frequent in people without Alopecia Areata. Eye strain was another suggested cause of Alopecia Areata (Kinnear 1939).
With the start of the twentieth century Alopecia Areata was known to be associated with disorders of the endocrine glands (Sabouraud 1913), particularly the thyroid. As such, some believed the underlying cause of Alopecia Areata was due to a hormone dysfunction. By the 1920's most dermatologists had abandoned the parasitic theory of Alopecia Areata and favored variations on the trophoneurotic and endocrine theories - often combining the two.
Sufferers of Alopecia Areata were certainly under extensive mental stress, not least from fear that they would be suspected of having ringworm or syphilis. Until the advent of antibiotics, syphilis was a widespread, contagious disease. It often manifests itself by sudden, rapid loss of hair in well defined patches, just like Alopecia Areata (Ormsby 1948, Roxburgh 1950), before progressing to development of lesions and sores. Syphilis in the secondary stage can also affect finger nails (Roxburgh 1950). To further complicate the matter, some dermatologists suggested that Alopecia Areata could be found in increased association with syphilis - as distinct from the direct action of syphilis on hair follicles. Syphilis was believed to induce Alopecia Areata by the mental distress it created and its possible upset of the endocrine system (Savill 1939). These clearly visible symptoms of syphilis were often confused with Alopecia Areata by the general population and resulted in social ostracism for the sufferer (Ormsby 1948).
The early 20th century saw the development of another hypothesis of Alopecia Areata induction based on toxic agents. An unknown poison was believed to be introduced to the hair follicle via the blood system inducing hair loss (Adamson 1912). The sudden remission and relapse of Alopecia Areata and its action simultaneously over the body was believed to support the idea. Also in support, injection of thallium acetate (rat poison) was shown to induce Alopecia Areata like hair loss in some people (Adamson 1912, Dixon 1927, Ormsby 1948), with expression of exclamation mark hairs - a diagnostic feature of Alopecia Areata (Roxburgh 1950). However, the toxic origin of Alopecia Areata never gained widespread popularity against the neuropathic and endocrine hypotheses and has long since fallen from grace.
It is now widely believed that Alopecia Areata is an autoimmune disease. Even though studies more than 100 years old showed that Alopecia Areata affected hair follicles were invaded by inflammatory cells (Giovannini 1891), the inflammatory autoimmune disease hypothesis did not become popular until the 1960s. The idea was first proposed by Rothman in a discussion of a paper by Van Scott (1958). Autoimmune disease occurs when a patient's immune system mistakes part of his or her own tissue for a foreign invading organism, attacks, and attempts to destroy, that part of the tissue. Increasingly, hard evidence is being produced in support of this hypothesis over the previous suggestions.
What are the initiating factors for Alopecia Areata?
We do not know what activates and promotes the onset of Alopecia Areata hair loss. There are several suggested factors that may influence the course of Alopecia Areata.
• 1) Psychologic long term chronic stress
• 2) Shock and sudden extreme stress
• 3) Physical trauma
• 4) Local skin injury
• 5) Genetic predisposition
• 6) Viral/bacterial infection
• 7) Pregnancy/hormones
• 8) Allergies
• 9) Chemicals
• 10) Seasonal changes
1) Psychologic long term chronic stress. A wealth of case-lore suggests that stress is an important precipitating factor in Alopecia Areata. Various reports tentatively support the stress hypothesis but so far it has only been shown by statistical correlation (Muller 1963, De Weert 1984, Perini 1984, De Waard Van der Spek 1989) - no direct physical link has been demonstrated. Indeed, some investigators refute stress being significant in Alopecia Areata (MacAlpine 1958). One of the problems with defining the significance of some journal reports on stress and Alopecia Areata is the lack control groups for direct comparison and evaluation. Further, the subsequent stress as a result of hair loss can confuse the issue in these inevitably retrospective studies. Stress is suggested as an environmental trigger in people predisposed to Alopecia Areata development (due to genetic susceptibility for example) rather than the primary basis for Alopecia Areata development (Muller 1963).
2) Shock and sudden extreme stress. There have been a number of reports on individual cases where clearly defined sudden stress events have preceded Alopecia Areata development. The emotional trauma of a family death or being involved in a car accident without major injury have been suggested as triggers for Alopecia Areata onset.
3) Physical trauma. There is a reasonable amount of case history evidence to show that physical trauma can trigger the onset of Alopecia Areata. Anything that stimulates the immune system from being hit on the head to an infection can be a potential trigger. Only recently has there been a demonstration of a tangible link between trauma and autoimmune diseases. Cells under physical stress can produce heat shock proteins (HSPs). As the name suggests HSPs are produced when cells are given heat shock. It has recently been found that cells also produce these proteins after exposure to other forms of stress such as inflammation, fever, irradiation, viral infection, malignancy, oxidation, heavy metals etc. The HSPs play a housekeeping role in immune system responses. These proteins have been implicated in the autoimmune diseases rheumatoid arthritis, lupus, and ankylosing spondylitis.
4) Local skin injury. Cuts, scrapes, and other abrasions of normal haired skin are often the focus for the onset of a new patch of hair loss in Alopecia Areata susceptible people. Ironically, similar abrasions in areas of skin already affected by Alopecia Areata can be the focus of temporary hair regrowth. Injury is known to promote anagen hair follicle growth in skin immediately surrounding the injured site.
5) Genetic predisposition. It has been shown that there is a higher incidence of Alopecia Areata occurring in genetically related individuals. This suggests that at least some people are genetically predisposed towards the development of Alopecia Areata. Several research groups have been examining the genetic make up of people who develop Alopecia Areata and found some genes to be much more common in people with Alopecia Areata compared to the general population. It is generally believed that Alopecia Areata susceptibility is polygenic - there are a number of genes which, if present, make that individual more likely to develop Alopecia Areata. The triggers for the actual onset of Alopecia Areata are most likely environmental but susceptibility to development of Alopecia Areata, the resistance of the Alopecia Areata lesion to treatment, and its persistence and regression and its extent over the body might be influenced by the presence and interaction of several genes.
6) Viral/bacterial infection. Cytomegalovirus infection of hair follicles has been implicated by at least one research group in development of Alopecia Areata (Skinner 1995). However, research by other groups has failed to confirm the potential link (Garcia-Hernandez 1998, Tosti 1996). HIV infection has also been suggested as a potential trigger for Alopecia Areata onset (Piras 1997, Grossman 1996, Cho 1995, Stewart 1993). Other dermatologists suggest general viral/bacterial infections may promote the immune system into an inappropriate response against hair follicles in susceptible people.
7) Pregnancy/hormones. The apparent link between hormonal fluctuations and Alopecia Areata has been recognized for some time (Sabouraud 1896, Sabouraud 1913). Most notable are the cases of Alopecia onset during late stage pregnancy. Women who already have Alopecia Areata can find that they have complete, but temporary, hair regrowth around the time of childbirth (Walker 1950). Puberty and menopause have also been suggested as a time of potential Alopecia Areata onset or remission.
8) Allergies. Statistical analysis shows that Caucasians with Alopecia Areata and some form of atopy (Asthma, eczema, rhinitis) are inclined to have hair loss that is more extensive and/or of prolonged duration (Muller 1963, De Waard Van der Spek 1989). Interestingly, statistical analysis of Indians with Alopecia Areata showed no such link (Sharma 1996). This may suggest that the different genetic composition of different races must be taken into account when explaining susceptibility to Alopecia Areata development.
9) Chemicals. One "outbreak" of Alopecia Areata in workers at a water treatment plant in a paper factory was linked to long term exposure to the chemical acrylamide (Roselino 1996). Formaldehyde and pesticides have also been suggested, although not proven, as a potential influence in the development of Alopecia Areata. Isolated case reports have suggested a link between Alopecia Areata development and Zidovudine treatment of HIV (Geletko 1996), and Fluvoxamine anti-depressive treatment (Parameshwar 1996).
10) Seasonal changes. A significant number of people with Alopecia Areata find the extent of the hair loss cycles in time with the seasons. Some people find the hair loss is much more extensive in winter and have temporary, partial regrowth in summer.
Whatever the initiation factor it need not be permanent - rather a short sharp shock may be just enough to tip the balance of the immune system into autoimmunity. Once an autoimmune disease is initiated it can be self perpetuating. Tissue destroyed in the early stages of the disease can be broken down and the antigens presented to immune system cells in the lymph nodes. This recruits more self reactive cells which destroy more tissue producing more antigens and so the cycle continues.
Diagnosis of Alopecia Areata
There is currently no conclusive diagnostic test for Alopecia Areata Dermatologists deduce Alopecia Areata by a process of elimination of other hair loss causes and close examination of the lesion itself. Typically, the initial Alopecia Areata lesion appears as a smooth bald patch sometimes within 24 hours. Some people feel a tingling sensation or pain in the affected area. The scalp is the most commonly affected area but Alopecia Areata can present in any region of hair on the body. Hair pull tests are sometimes conducted at the margins of lesions. If hair is easily pulled out, it is indicative that the lesion is active and further hair loss should be anticipated.
The hair fiber that falls out in Alopecia Areata has been the subject of several analytical studies and is sometimes used to diagnose Alopecia Areata. Using scanning electron microscopy the hair fibers falling out from the edge of an expanding bald patch can look very unusual. Frequently, the part of the hair fiber furthest away from the scalp (the oldest part of the hair) looks normal. Closer to the scalp (newer part of the hair) and it can look quite aberrant. We find the shape of the fiber becomes increasingly irregular the closer to the scalp we look. This involves deposits of unordered keratin and constrictions in the hair fiber. The cuticle can be missing and there can be longitudinal cracks along the length of the hair. Analysis of the hair fiber shows the constituent keratins to remain the same but the way they are assembled to make up the fiber becomes increasingly abnormal.
The irregular construction of the hair gives it weak spots where it can readily break off. This gives rise to the stumpy hair fibers called exclamation mark hairs that can often be seen in expanding patches of Alopecia Areata. The hairs are so called because they look like an exclamation mark [!]. Some dermatologists use presence of exclamation mark hairs as diagnostic for Alopecia Areata. However, it has been shown that exclamation mark hairs can occasionally occur in other conditions as well.
Anywhere between 7% and 66% of people with Alopecia Areata also have aberrant nail formation depending on which reports you read (Muller 1963, Baran 1984). It is generally agreed that 25% is a more realistic figure (around the mid point between 7% and 66%). Disruption of growth may involve all or just one of the nails. Nail dystrophy varies from a diffuse, fine pitting to severe alteration in a few cases (Gollinck 1990). For a more definitive diagnosis, dermatologists sometimes need to take a small skin biopsy (a small piece of skin about 4mm in diameter) for microscopic examination. With this, dermatologists can see whether there is focal inflammation of the hair follicles. This is the clearest method of identifying Alopecia Areata. More recently it has been suggested that a blood samples could be tested for the presence of hair follicle specific autoantibodies. However, while this is possible to do in a research or pathology laboratory no standardized test has been developed for the general dermatology clinic.
Subterms for Alopecia Areata
ALOPECIA AREATA (AA) - The most commonly used term and covers all forms of the disease.
ALOPECIA PARTIALIS - The name given to specify patchy hair loss. Not often used as "Alopecia Areata" suffices.
ALOPECIA TOTALIS (AT) - The name given to specify Alopecia Areata where all scalp hair is lost, but other body hair remains.
ALOPECIA UNIVERSALIS (AU) - The name given to specify Alopecia Areata where all scalp and body hair is lost.
ALOPECIA AREATA BARBAE - The term for an Alopecia Areata lesion found in the region of beard hair.
ALOPECIA AREATA OPHIASIS - The term used for an Alopecia Areata lesion limited to extension along the scalp margin (Occipital and temporal region - the strip of hair running from one ear around the back of the head to the other ear). Ophiasis comes from the latin "snake" due to the winding, snaking pattern the hair loss has over the back of the head. The term was originally used by Cornelius Celsus in 30AD.
ALOPECIA AREATA DIFFUSA - A term occasionally used for an Alopecia Areata lesion of general thinning of hair on the scalp. It is usually simply called diffuse Alopecia Areata.
RETICULAR ALOPECIA AREATA - The term used for the presence of numerous small, well defined patches of hair loss SISAIPHO ALOPECIA AREATA - This is a new term so far only used by one clinical group in Seville, Spain (Munoz 1996). It is defined as entire loss of scalp hair except for a narrow ring of hair around the periphery.
TRIANGULAR ALOPECIA AREATA - A very rare form of Alopecia Areata where the hair loss lesion presents in a triangular shape.
PERINAEVOID ALOPECIA AREATA - A very rare form of Alopecia Areata where hair is lost around nevi (moles or other skin growths).
(From Ormsby 1948, Muller 1963, Pecoraro 1963, Hordinsky 1988, Gollinck 1990, Rook 1991)
Synonyms for Alopecia Areata
These synonyms have largely fallen out of use.
• AREA CELSI
• ALOPECIA CELSI
• ALOPECIA CIRCUMSCRIPTA
• JONSTON'S ALOPECIA
• AREA JONSTONII
• PORRIGO DECALVANS
• TINEA DECALVANS
• CAZENAVE'S VITILIGO
• CELSUS' VITILIGO
• VITILIGO CAPITIS
NB Because of the arguments spanning several decades on the definition of Alopecia Areata some of the older terms have been used interchangeably with terms for other hair loss diseases. This is partly the reason why new terms have superseded older, potentially confusing, names for Alopecia Areata.
French synonyms for Alopecia Areata
The French never accepted the term Alopecia Areata and often use the term PELADE and sometimes TEIGNE PELADE, PELADE ACHROMATEUSE and PELADE DECALVANTE.
German synonyms for Alopecia Areata
Germans and German speaking populations typically use "Alopecia Areata" and its various subterms. Occasionally however, the German term KREISRUNDER HAARRAUSFALL is used which literally translates as "circle-round hair-outfall".