Circulatory alopecia

Circular alopecia (syn: circular alopecia, alopecia areata, nests baldness, pelada) - a disease characterized by the appearance of a rounded or oval center of alopecia with clear boundaries and externally unchanged skin. Limited forms of the disease can progress to complete hair loss on the scalp (total alopecia) and hair loss throughout the body (universal alopecia).

Patients with circular alopecia (CO) make up about 2% of dermatological patients. Representatives of both sexes are equally susceptible to this disease with a rise in incidence between the ages of 20 and 50 years.

Causes of Circular Alopecia

The cause of circular alopecia is still not established. A wide variety of concomitant diseases and unpredictability of the current make it possible to consider circular alopecia as a heterogeneous clinical syndrome, in the development of which the following factors play an important role:

1. Emotional stress by most authors is seen as an important trigger factor, at least for some cases of the disease. This view is based on clinical observations in which stress preceded the onset of circular alopecia and its recurrence, as well as the effectiveness of hypnotherapy and sleep treatment. An attempt to objectively assess the psychological status of patients showed the presence of abnormalities in 90% of patients, and in 30% of them, psychological disorders could be the cause of the disease or have a negative effect on its course. It should be noted. That the evaluation of the results of such studies is very difficult, since stress almost naturally occurs again due to hair loss. Patients with circular alopecia develop an inferiority complex, a tendency with introspection and a need for constant encouragement. Such a condition in psychiatric practice is referred to as dysmorphophobia, that is, fear of losing its habitual appearance, which should be taken into account when prescribing therapy.
2. Infection. There are cases of the appearance of circular alopecia after acute infectious diseases. Many, mostly domestic, scientists recognize the role of foci of chronic infection (carious teeth, periapical granulomas, sinusitis, frontalitis, otitis, etc.). However, there is still no reliable evidence that their combination with circular alopecia is not accidental.
3. Physical trauma, like infection, can be a potential cause of the disease. Under the influence of physical stress, cells can produce heat shock proteins that play an important role in the developed immune response.
4. Genetic factors. The frequency of circular alopecia in a family history is 4-27%. There are reports of a circular alopecia in twins, with some couples developing the disease at the same time. An opinion is expressed about the autosomal dominant type of inheritance with different penetrance of the gene. The role of racial differences is not excluded: among Japanese inhabiting the Hawaiian Islands, circular alopecia is a frequent disease.

The combination of circular alopecia with diseases of the atopic circle (atopic dermatitis, bronchial asthma) has been studied since 1948. The frequency of this combination, according to different authors, ranges from 1% to 52.4%. Japanese doctor T. Iked identified 4 types of circular alopecia, among which atopic occurs most unfavorably, in 75% of cases leading to total baldness.

The study of associations of circular alopecia with the genes of the main histocompatibility complex (HLA), as well as the results of polymorphism of interleukin 1 receptor antagonist genes, testify to the genetic heterogeneity of this disease, which may explain the clinical polymorphism of alopecia are well known to physicians.

Pathogenesis of circular alopecia

Most clinicians support the hypothesis of an autoimmune nature of circular alopecia. The search for arguments confirming the hypothesis is conducted in three directions: the identification of combinations with autoimmune diseases, the study of the humoral and cellular links of immunity.

Combination with autoimmune diseases. Most often there are descriptions of a combination of circular alopecia with thyroid gland diseases, but the figures that characterize its frequency vary widely (8-28%). There are numerous reports of cases of a combination of circular alopecia with pernicious anemia, vitiligo, systemic lupus erythematosus, scleroderma, rheumatoid arthritis, autoimmune testicular pathology and many other autoimmune diseases.

It is known that patients with Down's disease are susceptible to various autoimmune diseases. Circular alopecia in these patients occurs 60 times more often than in other mentally retarded people. Almost half of patients with Down's syndrome have a total or total alopecia.

The state of humoral immunity. The study of various organ-specific autoantibodies produced conflicting results, which can be explained by the comparative smallness of the patients surveyed, and by the differences in the survey methods. Thus, antibodies to microsomal structures of the thyroid gland, smooth muscles, parietal cells of the stomach, antinuclear antibodies and rheumatoid factor were detected in the serum of patients with circular alopecia. It is pertinent to recall that a low level of autoantibodies that do not have any damaging effects is considered the norm and is found in most people.

The first direct indications of the possibility of autoimmune mechanisms of circular alopecia have been studied in recent years with the advent of new facts, indicate the presence of autoantibodies to hair follicles in 90-100% of patients with alopecia areata, and the level of the detected antibodies was significantly higher than in control.

Moreover, various autoantibodies of classes IgM and IgG to several antigens of hair follicles have been found out.

State of cellular immunity. Controversial data were also obtained in the study of the cellular link of immunity. The total number of circulating T cells is characterized as reduced or normal; the number of T-suppressors is reduced, normal and, even, elevated. Various functional disorders of T-lymphocytes are also revealed.

Direct evidence of the autoimmune genesis of circular alopecia is the detection of lymphocytic infiltrates inside and around the hair follicle, as well as accumulations of Langerhans cells in the peribulbar area. When treating patients with contact allergens or minoxidil during hair regrowth, the number of T cells in the peribulbar zone decreases, and if the therapy remains ineffective, it remains the same.

Attempts to identify antibodies against the components of the follicle in the skin of the scalp have not been successful.

In active centers of circular alopecia, expression of HLA-DR antigens on the epithelial cells of the pre-cortex matrix and the hair vagina was detected; this is considered as a mechanism by which cells present their specific surface antigens to sensitized T-inducers.

Thus, circular alopecia appears to belong to the group of organ-specific autoimmune diseases, as evidenced by a hereditary predisposition, an increased frequency of detection of organ-specific antibodies, and a violation of the T-cell regulation of the immune response. However, since the antigen concerned is not established, it remains unclear whether the normal components of the hair (melanocyte, ksratinocyte, cells of the papilla) are affected or the immune system responds to pre-damaged hair follicle tissues. In addition, unlike most autoimmune diseases, with circular alopecia to date, it has not been possible to detect antibodies against follicular components in the scalp. The search for such evidence is extremely promising.

If such evidence is presented, circular alopecia will become unique among autoimmune diseases due to non-destructive changes in the target organ.

It should be mentioned that a small part of dermatologists challenged the autoimmune genesis of circular alopecia without denying the immune mechanism of the disease. The basis for this opinion was the identification of the genes encoding the cytomegalovirus (CMV) in the skin, while in healthy people the expression of these genes was not detected. The authors believe that the presence of CMV in hair follicles causes an immune response, leading to tissue damage. This hypothesis certainly needs proof, but the possibility of the origin of the target under the influence of an external source is not refuted by itself.

Pathophysiology and Pathomorphology

It was established that circular alopecia begins with the premature entry of follicles into the telogen phase in the center of the emerging focus, followed by a centrifugal spread of the process in the form of a divergent wave. The ratio of anagen and telogen hair varies widely, depending on the stage and duration of the disease (normal A / T = 9: 11). As the results of histological examination showed, in the early stage of circular alopecia most follicles are in the telogen phase or late catagen; a few follicles in the anagen phase are located in the dermis at a higher than normal level. The development of the hair follicle with circular alopecia stops in the phase of anagen III, when the inner root vagina takes on a conical shape, and the differentiated cortical cells show no signs of keratinization. An exceptionally significant histological feature is the presence of a dense peribulbar intraphollicular lymphocytic infiltrate, more pronounced in the early stages of baldness and consisting predominantly of T-cells and Langerhans cells. Sometimes the infiltrate also captures the upper, unchanging part of the hair follicle in the phases of the anagen or telogen. As mentioned above, the infiltrate is resolved when hair growth resumes. The number of hair follicles in the formed hearth decreases. The secretory activity of the sebaceous glands decreases with an increase in the duration of the disease. Sometimes a prolonged course of the disease leads to the death of the hair follicle and irreversible hair loss; it is possible that in these cases the pathogenetic mechanisms coincide with those in a pseudo-peloid. Histological examination of the affected skin helps detect atrophic changes.

Characteristic for circular alopecia violations of the structure of the hair shaft are well known. Pathognomonic sign are the hair in the form of exclamation marks, which, however, are not always present. It is a hair of a clavate shape about 3 mm long. The distal end of these hair is split; down from the tip of the hair the cone-shaped thickens, the bulb of the hair is reduced in size, but in other respects it is normal. When hair growth resumes, follicles are produced that produce several thin rods.

A large contribution to the study of pathological changes in the follicle was made by a group of scientists led by A. Messenger. It has been shown that keratinocytes in the keratogenic zone are damaged in the focus of circular alopecia in the anagenic follicle. With the help of electron microscopy, the fact of nonspecific damage of matrix cells over the upper pole of the dermal papilla as well as the cells of the keratogenic zone was established. Expression of HLA-DR antigens was detected in the cells of the pre-cortex matrix and keratogenic zone, suggesting that it is these parts of the follicle that are the primary target for circular alopecia. The authors proposed a hypothetical model explaining the formation of hair in the form of exclamation marks and the non-destructive nature of the disease.

The essence of the hypothesis is that, in response to damage, depending on its severity, follicles can react in three different ways. Severe injury damages and weakens the hair in the keratogenic zone, which forces the follicle to go into the catagen phase, and then the telogen. Such hair breaks off when their keratogenic zone reaches the surface of the skin. It is these hair that later resemble exclamation marks. Another follicle can go on time to the normal catagen phase and then telogen and fall out with a normal bulavate bulb. Such follicles in the new cycle produce dystrophic hair. And finally, some follicles are probably damaged so insignificantly that, Despite the appearance of dystrophic changes, the anagen phase is not interrupted.

Symptoms and course of circular alopecia

The disease begins with the sudden appearance of a rounded focus of alopecia, which accidentally notices either the patient himself, or (the cup) - his relatives or a hairdresser. Subjective sensations, as a rule, are absent, however some patients note the increased sensitivity of the skin or paresthesia, preceding the origin of the focus. The boundaries of the hearth are clear; the skin within it is smooth without inflammation and peeling, sometimes a testy consistency and is easier than it is healthy in the folds; the mouth of the hair follicles are preserved. Sometimes in the initial stage of alopecia the skin is slightly hyperemic. In contrast to the pseudo-pellet, there is a lack of skin atrophy and individual hair bundles in the center of the focus of alopecia. In the progressing stage healthy looking hair at the edges of the hearth is easily epilated; characteristic of the appearance of the ox in the form of exclamation marks. The further course of the disease is unpredictable. Sometimes within a few months the growth of hair in the hearth is completely restored. New foci may appear at different time intervals. Individual foci can quickly merge due to the diffuse loss of hair that separates them. Perhaps diffuse hair thinning without forming foci of alopecia. There are cases when the disease began with a diffuse hair loss and led to total baldness within 2 days. The resolution of one of the foci can be combined with a progressive loss of hair in another hearth. Collateral circular alopecia arising after trauma is described.

In 60% of cases, the first foci occur on the scalp. It is also possible hair loss in the area of growth of the beard, especially noticeable in dark-haired men. In many cases, circular eyebrows drop eyebrows and eyelashes, sometimes this is the only manifestation of the disease. It is possible to partially or completely lose the tufted hair on the trunk and hair fall out in the axillary and pubic regions.

Gray hair with circular hair loss is usually not involved in the pathological process. If gray hair dominates, then with the sudden dropout of all pigmented hair, a false impression may appear that the person has turned gray for several days. The newly growing hair is at first thin and devoid of pigment and only gradually acquires normal thickness and color. Bunches of growing gray hair resemble a picture of a polio. The facts suggested that melanogenesis is the target for circular alopecia. Concerning the fate of the melanocytes themselves in the affected hair follicles, various opinions are expressed: some authors note their disappearance, others manage to detect them. Pigmentary disorders in the growing hair are probably due to incomplete melanocyte activity in the early anagen. It was established that the activity of melanocytes correlates with the differentiation of cortical cells, and, perhaps, depends on it. The opinion is expressed that circular alopecia is a disease of differentiated cortical keratinocytes, therefore the follicle, which is in the telogen phase, is involved in the pathological process; This also explains the non-destructive nature of the disease.

Eye changes. Disorders of pigment formation with circular alopecia can affect not only the melanocytes of the hair follicles, but also the pigment cells of the eyes (changes in the color of the iris from brown to blue, spotty atrophy of retinal pigment epithelium, pigmentary hyperplasia, hyperpigmentation and hypopigmentation of the retina, etc.). Changes in the pigmentary system of the eyes with circular alopecia are similar to those in vitiligo. The relationship of circular alopecia and cataracts is debated.

Nail changes occur in 10-66% of patients suffering from circular hair loss. Dystrophy of the nail plates can be manifested by a variety of changes: point depressions, thinning and brittleness, longitudinal striation, coilonichia (spoon-like concave nails), thickening of the nails, onycholysis (partial separation from the nail bed), onychomadez (total separation from the nail bed).

Classification of circular alopecia

A single classification of the disease does not exist. Depending on the area of the lesions, the following clinical forms of circular alopecia are distinguished.

Focal alopecia is characterized by the appearance of one or several large, up to several cm in diameter, foci of alopecia on the scalp or in the area of growth of the beard. Within a few months, the growth of hair in the hearth can be fully restored. In case of unfavorable course of the disease, alopecia areata can become subtotal, total and universal.

Subtotal alopecia is diagnosed with small hair growth areas remaining on the scalp; for total characteristic the complete absence of hair on the scalp. Universal (malignant) alopecia is characterized by a lack of hair in all places of hair.

Obviously, the above classification lacks quantitative parameters for the evaluation of the area of the lesion, which greatly complicates the comparative evaluation of the published clinical data. Filling this obvious gap, American dermatologists with years of experience researching the problem (Olsen E. Et al.) Proposed criteria for quantifying the degree of alopecia. The authors focus on the state of terminal hair on the scalp, taking into account the main clinical forms of the disease (focal, total, universal).

Several methods for estimating the area of alopecia have been proposed:

1. Mentally divide the scalp into 4 quadrants. Calculate the percentage of total area of alopecia. The area of each quadrant is 25% of the scalp area.
2. If the total area of all sites is 100%. For example, if the hair is missing by 1/4 (25%) of the occiput, the area of the entire scalp is 0.25 x 24% = 6%. If in the same patient the second focus of alopecia occupies 40% of the vertex, this will be 0.4 x 40% = 16% of the scalp area. Thus, the total area of alopecia in this patient is 6% + 16% = 22% of the scalp, or S, according to the proposed classification.
3. With subtotal alopecia it can be easier to assess the area of the scalp with the preserved hair. For example, hair growth is maintained at 8% of the scalp area; hence the total area of the lesion center is 92% (S4a).
4. It's easy to draw pockets on the diagram; this method makes it easier to document the location and size of the outbreaks. If the foci are numerous and scattered, it is convenient to use the image analyzer to determine the area of the lesion.

Each physician has the right to use the method that seems more convenient to him, but the chosen method should become the standard in assessing the degree of scalp damage in all patients in this particular study.

S (scalp). Hair loss on the scalp.

• S0 = hair saved
• S1 = 25% of hair loss
• S2 = 26% -50% of hair loss
• S3 = 51% -75% of hair loss
• S4 = 76% -99% of hair loss
  • Sa = 76% -95% of hair loss
  • Sb = 96% -99% of hair loss
• S5 = 100% hair loss

In the (body). Hair loss on other parts of the body.

• B0 = hair saved
• B1 = partial hair loss
• B2 = 100% hair loss

N (nail). Change of nail plates.

• N0 = none
• N1 = partially changed
• a = dystrophy / trachionychia of all 20 nail plates

Terminology:

Alopecia totalis (AT) = S5B0

Alopecia totalis / alopecia universalis (AT / AU) = S5 B0-2. The term is recommended for use in total alopecia, accompanied by partial hair loss on the trunk.

Alopecia universalis (AU) = S5B2.

With subtotal alopecia of the scalp, as well as in the presence of foci of hairy or bristly hair, the terms AT, AT / AU and AU are not applied.

According to the authors of the classification, the use of these standards will make the evaluation of clinical data more objective, which will facilitate the cooperation of doctors studying the problem of circular alopecia.

In addition to the forms of the disease, characterized by the area (and, consequently, the severity) of the lesion, two more clinical varieties of circular alopecia are distinguished:

Ophiasis (serpentine, ribbon-like form) is manifested by hair loss in the occipital region and the spread of the lesion along the periphery of the scalp to the auricles and temples. This form of alopecia often combines with the atonic state and is highly torpid in the therapy.

Spot (mesh, pseudo-syphilitic) form of the disease is characterized by the emergence of a network of small, a few mm in diameter, contacting foci of hair loss scattered throughout the various parts of the head. Like the previous one, this form of circular alopecia is prognostically unfavorable.

Of great interest is the pathogenetic classification (T. Ikeda), which takes into account the concomitant clinical pathology and the prognosis of the disease. The author identifies 4 main types of circular alopecia (the frequency of cases characteristic of Japan is given).

• Type I. The usual type. Characterized by the emergence of round foci of alopecia. It occurs in 83% of patients, occurs mainly in the age of 20 to 40 years and ends in less than 3 years. In some foci hair grows in the first 6 months. Total alopecia develops only in 6% of cases.
• Type II. Atopic type, occurring in 10% of patients. The disease occurs in children suffering from bronchial asthma, atopic dermatitis or polynomia, and is characterized by a mesh pattern of hair loss or the appearance of individual rounded foci. Individual foci usually last more than a year. The total duration of the disease is up to 10 years or more. Total alopecia occurs in 75% of patients.
• Type III. Prehypertensive type (4%) occurs mainly in young people whose parents suffer from hypertension. It is characterized by a rapid current, a mesh pattern of hair loss. The incidence of total alopecia is 39%.
• Type IV. Mixed type (3%); the onset of the disease is older than 40 years, the course is long, but in total alopecia passes only in 10% of cases.

In general, this classification was approved by scientists in a number of countries, although the author's identification of a prehypertensive type of disease was not supported

Thus, circular alopecia is characterized by a variety of clinical forms combined with inherited and autoimmune pathology, infectious diseases; the role of environmental factors is not excluded.

Despite the unpredictability of the course of circular alopecia, it can be argued that the prognosis of the disease is worse in the appearance in the pre-pubertal period, especially in the presence of atopy, in ophiose, as well as in detecting high titers of antibodies to the components of the thyroid gland and leukocyte nuclei. Although initially the appearance of circular alopecia is not associated with atrophy of hair follicles, the long course of the disease can gradually lead to dystrophic changes in follicles and their death. This process, as with a pseudo-pelvis, is not accompanied by visible inflammation of the skin. Histological examination helps to identify the formed atrophic changes.

Diagnosis of circular alopecia

Diagnosis of circular alopecia usually presents no difficulties. On examination, one should be convinced of the absence of inflammatory erythema, ecdysis, atrophy, telangiectasias and other skin changes. The diagnosis can be verified by examining the hair, which in the progressing stage is easily epilated from the zone surrounding the outbreak. In the zone of shaky hair, telogen and dystrophic hair, as well as hair in the form of an exclamation mark, are easily identified when viewed with a magnifying glass or under a microscope at low magnification.

It is also necessary to inspect the area of growth of the beard, mustache, eyebrows, eyelashes and the entire skin for the detection of pockets of circular alopecia left unnoticed by the patient. You should pay attention to the condition of the nails, as their dystrophic changes are considered a prognostically unfavorable sign.

Given that the success in treatment depends on how fully identified and corrected the likely etiological and pathogenetic factors, the patient with a circular hair loss should be carefully examined.

Particular attention should be paid to the search for foci of chronic infection, primarily odontogenic and ENT organs, for which the use of X-ray diagnostic methods (orthopantomogram, Rg-gram of paranasal sinuses). It is also necessary to have an ultrasound of the abdominal organs and, in women, a small pelvis. The survey and evaluation of the results are carried out with the participation of specialists of the appropriate profile.

To identify other intercurrent diseases and disorders, it is necessary to evaluate the hemogram, the biochemical parameters of the blood, the coagulogram, the thyroid gland metabolites and the adrenal cortex, the immune status study, the Rg-gram of the Turkish saddle, the EEG. Many patients need the consultation of an endocrinologist, and women - and a gynecologist-endocrinologist.

Differential diagnostics

First of all, it is necessary to exclude cicatrical baldness, or the condition of the pseudo-pelvis, which is the final symptom of a number of skin diseases on the scalp. The surface of the skin in the areas of alopecia with pseudapelade is smooth, white, shiny, without skin pattern and the mouth of the hair follicles. Atrophied areas somewhat sink, not compacted. Within the foci, individual hair or hair bundles may be retained.

Mycosis of the scalp should be ruled out in the presence of ecdysis, hyperemia, broken hair (including low-bruised - "black spots"), infiltration and cicatricial focal alopecia. For this purpose, inspection under a mercury quartz lamp with Wood filter and mycological examination of altered hair and scales are used.

The presence of a large number of small, 1-1.5 cm in diameter, incorrectly shaped foci of rarefaction of hair, reminiscent of "fur, eaten by a moth" should suggest secondary syphilis; in such cases, other clinical manifestations of this disease should be investigated and serological examination of the blood should be carried out.

Trichotillomania - a neurotic state in which the patient himself pulls out his hair - can present certain diagnostic difficulties. With trichotillomania, foci of alopecia are bizarre contours, with uneven contours, while retaining part of the hair within their boundaries. Dystrophic hair and hair in the form of exclamation marks are absent, as there is no zone of shaky hair.

The acute onset of diffuse hair loss with circular alopecia is difficult to differentiate from the diffuse telogen hair loss that occurs after taking a number of medications, x-ray therapy, poisoning with mlschak, mercury, etc. Violation of the hair cycle can also result from infectious diseases accompanied by fever (above 39 ° C) intoxication (secondary syphilis, HIV infection, etc.). The diagnosis of circular alopecia is confirmed by the presence of dystrophic hair and hair in the form of exclamation marks. In all cases of diffuse hair loss, a serological test is needed to exclude syphilis and HIV infection

Alopecia areata can be official and arise as a result of excessive hair extensions when winding on hair curlers, curling hot tongs, pulling a hairdo ("ponytail"), etc.

Pronounced alopecia can develop with congenital dystrophies of the hair shaft (monitriks, trichotortosis, etc.), which are detected at the birth of a child or develop during the first years of life. Correct diagnosis of these rare diseases contributes to anamnesis, the detection of broken hair and the identification of defects in the rod with a careful microscopic examination. With circular alopecia, there is no change in the hair shaft.

Treatment of circular alopecia

So far, there has not been found a universal, safe for human medicine, which would permanently save the patient from the circular alopecia.

Thus, reports on the high effectiveness of various drugs in the treatment of the usual type of baldness (T. Ikeda classification) should be treated very critically, since even without treatment the disease is prone to independent remissions, and only 6% of patients develop total alopecia. At the same time, atopic type of circular alopecia, despite treatment, total alopecia occurs in 75% of patients. The real effectiveness of the agent used can be demonstrated only by a stable success in the treatment of total and universal alopecia - traditionally resistant to therapy forms of circular alopecia.

Experience shows that torpidity for therapy and an unfavorable prognosis are possible under the following circumstances:

• family history of the disease
• concomitant atopic state
• combination with autoimmune diseases
• onset of the disease before puberty
• frequent relapses
• ophyza, total and universal forms of circular alopecia
• combination with severe dystrophic lesion of nail plates
• prolapse of newly growing loose hair follicles

Therapy should be comprehensive and as individual as possible. The purpose of treatment should be preceded by a thorough examination of the patient to identify and correct for concomitant diseases and background disorders (foci of infection, psychogenic factors, neurotransmitter, microcirculatory and hemorheological changes, hypertension-hydrocephalic syndrome, etc.).

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