Services on Demand
On-line version ISSN 1806-4841
An. Bras. Dermatol. vol.79 no.5 Rio de Janeiro Sept./Oct. 2004
José Marcos Pereira
Ex-professor of Dermatology at the Faculdade de Medicina da Santa Casa de Sao Paulo
Due to its aesthetic importance, our hair is often a target of social manifestations, as in the case of bizarre hairstyling and hair dyes and sometimes even self-aggression. The most frequent acts of self-aggression are: trichotillomania, trichotemnomania, trichophagy, trichoteiromania, scratching-pseudoalopecia, trichocryptomania, trichorrexomania and neuropathic plica. In this article, the author discusses in detail the clinical characteristics and propedeutics of each disease, with emphasis on the psychiatric aspect of the patients.
Key-words: alopecia; hair; trichotillomania.
In all cultures and throughout all the ages the hair has been highly regarded. Many works have described the psychological and social trauma that a person suffers upon losing his/her hair. An interesting study was done by Maffei and cols.,1 that compared the emotional behavior of patients with androgenic alopecia with that of the population with normal hair. They reported that in the sampling with normal hair, 10.3% had some psychiatric disturbance, such as paranoia, antisocial behavior, obsession, aggressiveness, sadism, and depression, among others. In patients with androgenic alopecia, the level of disturbance reached 76.3%.
The hair, without any doubt, is the most significant ornament for the human being. It is clear that, due to its aesthetic importance, hair becomes the most obvious means of cultural expression, such as with haircuts, hair styles and bizarre colorations, such as for example, dreadlocks and black power, etc. The hair is also the target for self-aggressive behaviors, or the so-called compulsive trichoses. The best known compulsive trichosis in dermatology is trichotillomania, however there are others less well known that should be kept in mind, such as trichotemnomania, trichophagy, trichoteiromania, scratching- pseudoalopecia, trichocryptomania, trichorrexomania and neuropathic plica, each of these with its own characteristics.
Trichotillomania is the compulsive action of pulling out the hair, and may involve hair of any area of the body. In attending a patient with trichotillomania, great caution is required, since this should be done considering the psychiatric as well as the dermatological point of view.
1. Psychiatric aspects
Compulsive action is a psychopathic disturbance characterized by a repetitive and uncontrollable impulse to do a particular act.2 The clinical characteristics of a compulsive action may be defined as: a failure in the attempt to resist the impulse; a temptation to inflict dangerous acts against oneself or others; an increase in tension or excitement immediately before the action; and a gratification or pleasure soon after the action. Of the wide variety of impulses, four are the most frequent: compulsive gambling (for instance in a casino), kleptomania, trichotillomania and compulsive shopping.2 Psychiatric studies have created criteria for the diagnosis of trichotillomania. According to the Diagnostic and Statistical Manual of Mental Disorders (DSM-IV),2,3 trichotillomania should be considered a compulsive disorder and requires the following criteria for diagnosis: 1) recurrent traction of the hair, causing a perceptible alopecia; 2) an increase of a sense of tension immediately before the action of hair pulling or when trying to resist the impulse; 3) pleasure, gratification or relief when the hair is pulled and consequently extracted; and 4) mental illness should not be considered the only explanation for the existence of this disease. Obviously, these criteria of the DSM-IV, except for the first, are psychiatric evaluations that do not lead to a definitive diagnosis. As for the first item, it is quite vague dermatologically, since trichotillomania when presented in the clinic is only one of a very wide range of clinical possibilities, thus even the most experienced dermatologist may be deceived. Besides this, when the trichotillomania assumes a form more or less diffuse, the rarefaction is only noticeable after a loss of 30% of the hair.3
When the trichotillomania occurs after adolescence it is more probable that there exists a psychopathic disorder that causes anxiety.3 Women are much more affected than men. Surprisingly, many times these patients do not worry about the sometimes unkempt appearance of their hair (la belle indifférence). This constitutes a strong indication for the diagnosis of this condition. Many patients openly admit that they pull their hair, however most categorically deny any manipulation of their hair, thus hindering a therapeutic approach.4 Trichotillomania in adults is more serious than in children, amongst whom, the situation often resolves spontaneously with the passing of time.5
Some authors estimate that in the USA alone two to eight million persons may have trichotillomania, of these 90% are women who should have their psychosocial aspects properly analysed.6
Enos and col.7 created guides for diagnosis, for measurement of intensity and for the management of trichotillomania.
Trichotillomania may occur in any pilose area of the body, such as the beard, eyelashes, pubic hair, etc.,8 however this article focuses only on trichotillomania of the scalp.
Trichotillomania has a prevalence of 0.6% among students, with a reported incidence of 3.4% in women and 1.5% in men. Many times trichotillomania passes unnoticed. In general, 40% of these cases are not diagnosed, and 58% of the patients are not treated.2
Studies in closed populations show that, when trichotillomania is actively investigated, or that is, going beyond counting specific complaints or of patients seeking medical help, its incidence is found to be much greater.9
From the dermatological point of view, trichotillomania is very rich in propedeutical signs. The situation is occasionally triggered or worsened by a pathology in the scalp, such as eczema, neurodermatitis, dermatophytosis or even alopecia areata. It may be followed by trichophagy,10 and at times hair may be found inside the patient's mouth or accumulated in the stomach or intestine, forming the classic trichobezoar.
Usually aggression to the hair occurs when the patient is watching television, studying, talking on the phone, etc., and is intensified when associated with a stressful situation, for example when preparing for an examination. The extraction of the hair is not always immediate. Often the patient spends hours in twisting the hairs with the fingers or manipulating them in some way and only later actually extracting them. This is occasionally done with tweezers.3,11 The extraction of anagenous hairs is more difficult and painful, for this reason telogenous hairs are usually pulled out first.11 Trichotillomania may have alternating periods of exacerbation and relative calm.8
A patient with trichotillomania, adult or child, rarely wants to consult a physician. Generally, it is not considered important in spite of the often bizarre appearance of the hair. In most cases the patient goes to the clinic with a companion who has taken a greater interest in the disease. Usually the patient has already been seen by other doctors and consequently neither the patient nor the companion want to accept or admit the hypothesis of a trichotillomania. In spite of great tact on the part of the physician in approaching the subject, the great majority of patients never return after confirmation of the diagnosis.
The clinical presentation of trichotillomania is extremely variable, in that in general, it may involve any of several areas in the temporoparietal region, a single lesion is rare. A standard pattern for alopecia does not exist. The picture may be radical and bizarre or even so discreet as to the point of passing unnoticed. Rarely will an area be observed with complete alopecia, that is, with total absence of hair. Usually there is a thinning with many broken hairs and many others growing again. Sometimes, because of the constant manipulation, the hairs are curled irregularly, assuming the appearance of the kinking syndrome, or even the syndrome of unmanageable hair. In some cases excoriations or small wounds may be found in the scalp. Eventually these may even form a plaque of neurodermatitis localized in the area of alopecia.
In the affected area, hairs of several types may be observed: shafts with normal ends; some in flammule, showing that they are "sprouting" hairs; many of several sizes with extremities in a brush shape, characterizing a breaking off; and some that are curled. Many follicles are empty, and some present darkened impregnations, which are the remains of stems (trichomalacia). The classic exclamation mark hair is not pathognomonic of alopecia areata. In trichotillomania, hair quite similar to that of the exclamation mark hair may also be found. In alopecia areata, exclamation mark hair forms a brush at the distal extremity, as in the split hairs of trichorrexis nodosa. However, in trichotillomania the distal extremity is cut off cleanly, or that is the ends are smooth and rounded.12
Also in the dermatoscopy it is common for trichoptilosis, pili torti and tricorrexis nodosa to be observed.
The Gentle Traction Test
Since most of the hairs are in the anagen phase, they do not come loose easily with this test, thereby demonstrating a negative result. However, as there may be a lesion of the stem, the hair may break and come loose, leading to a false positive result as they are actually pieces of the stem.13 In these cases it is fundamental to use a simple optical microscope to analyze the extremities of the hair shafts thus obtained.
The Hair Pull Test13
This test is done by an intense traction on the hairs. It is a useful test because if the shafts in general are brittle, the obtaining of fragments demonstrates the fragility of the hair.
The Attrition Test
The attrition test consists of rubbing with the index finger a wad of hair placed in the center of the palm of the other hand.13 Due to the constant lesions of the hair shafts, on performing the attrition test, broken pieces of hair are observed in the palm, thereby characterizing lesions of the stems.
The trichogram in trichotillomania can be quite characteristic.13 Since the telogenous hairs are the most easily extracted as they have less adhesion to the follicles and their extraction is less painful, thus the anagen hairs are left in the scalp, thus the trichogram can reach 100% anagenous hairs.11 Besides this, every capillary replacement is anagenous, which contributes to the increase in anagenous hair.
Analysis of hair eliminated spontaneously
When the patients extract their hair, they have three options as to what to do with it: 1) eat the hair (trichophagy); 2) hide or dispose of it, for instance, in the garbage, flush it down the toilet, etc.; and 3) simply let it fall to the ground, wherever they are. Considering these possibilities, there are two convenient ways to make the collection of samples in a case of trichotillomania. Firstly, the most important and reliable method is a collection done by a companion of the patient, usually a relative, who is asked to find and collect the hair in the environment in which the patient lives. Careful attention is required to collect hair on the ground, on the desk where the patient studies, in the bathroom, on the pillowcase, etc. It is important that care be taken to not let the patient know about the collection, because his or her reaction might be to temporarily stop extracting the hair or simply to hide it. The second form of collection is done by the patients themselves. Generally though, these patients do not show much interest in cooperating with the doctor, almost always bringing in very small amounts of collected hair.
When the hair is analyzed from either means of collection, the presence of anagenous, catagenous or telogenous hairs with epithelial sacs shows that the hairs were extracted by traction, which confirms trichotillomania.13 The presence of broken hair is suggestive, but does not confirm the diagnosis.
Study of the pelage
When examining a patient with trichotillomania, a study of the pelage may clarify some aspects. These are fine hairs, usually only a few millimeters in length, observed with the aid of a paper card held perpendicular to the scalp.13 A sample of them is removed with tweezers and observed under a simple optical microscope.
When the patient extracts a hair shaft in its anagen phase, three possibilities may occur: the hair breaks off near the matrix, in which case it will soon continue to grow; fragmentation of the hair occurs inside the follicular channel, which may cause the formation of a trichomalacia; and finally, the hair breaks off a few centimeters from the surface of the scalp. In all three possibilities, the pelage to be found is anagenous and with splintering of the distal extremity.
When the extracted hair is telogenous, it rarely breaks as its adhesion to the follicle is weak. Its replacement will be with an anagenous hair that will have as its characteristic a thin distal extremity in flammule. These concepts are important, because if the patient has a disease associated with trichotillomania, such as, for instance, an androgenic alopecia, there will be predominantly telogenous pelage.
A exclamation mark hair is still part of the pelage, and its shaft is very characteristic since its bulb is generally telogenous, although in rare cases it may be anagenous.
The Fluorescent Light Test
This simple test shows whether or not the patient actually manipulates the scalp. Close to the altered area of the scalp, fluorescent ink of the type used in text marking pens is applied. The following day the patient's hand is examined with a Wood lamp.13 Fluorescence on the hands shows that the patient had manipulated the hair.
In many cases the value of this test is mainly to convince the companion of the patient that manipulation of the hair is actually taking place.
A small area of hair is cut close to the scalp and isolated with adhesive tape.13 This test is a very useful resource, and perhaps the best indication for this type of test is to demonstrate to the patient and companions that the hair is growing normally.
Histopathology is important for confirming the diagnosis14 or, at least, for ruling out other diseases. Classically, several empty follicular channels will be found, as well as some follicles with dilated infundibula with corneous plugs. Most of the follicles will be in the catagenous phase or initial anagenous, few will be telogenous. The complete lack of perifollicular processes will be notable. Some follicles are destroyed by perifollicular hemorrhage, by pigmentary remains close to the isthmus of the follicle and by the characteristic trichomalacia caused by pieces of hair within the pilose follicle.
Trichophagy is the compulsive action of swallowing hair. Patients with long hair, who may be in the habit of putting wads of hair in the mouth and chewing, could be swallowing some of the pieces. Another form consists of the patient pulling out the hair, characterized as trichotillomania and thereupon transferring it into the mouth.
Whenever faced with a clinical picture of trichotillomania, the possibility should always be considered that the patient may be swallowing hair, and that this could lead to a trichobezoar.15 When examining a patient with trichotillomania, an examination of the mouth should not be overlooked because fragments of hair might be found between the teeth or adhering to the mucous membrane.
At least theoretically, any other compulsive trichosis, such as trichotemnomania, trichoteiromania, scratching-pseudoalopecia, trichocryptomania and trichorrexomania, may lead to a trichophagy, which in turn may result in the formation of a trichobezoar, also known as Rapunzel's syndrome.15 This is defined as a mass of hair that is formed in the stomach or, more rarely, in the small intestine,16 resulting from the ingestion of hair. Usually the patients present a profile of trichotillomania, although cases of trichobezoar formation have been reported in which the patient did not present alterations in the scalp. Most of the cases (90%) occurs among girls with long hair.16 Usually these patients are children with weight loss and imprecise gastrointestinal manifestations. Clinically, vomiting and nausea can be observed in 64% of the patients, abdominal cramps in 70%, alterations of the intestinal habits in 32%, palpable epigastric mass in 88%, as well as other gastrointestinal alterations. Without early diagnosis and treatment, consisting of either removal by endoscopy or as a last resort surgery, it can lead to gastric necrosis, hematemesis or intestinal perforation with peritonitis. The mortality rate can reach 50% .16 Diagnosis by x-ray and ultrasound scan may suggest a gastric mass, but endoscopy provides confirmation of the diagnosis.
Most of the cases of trichobezoar are associated with mentally retarded persons.17
The term trichotemnomania was created by Braun-Falco and col.,18 in 1968, to describe the case of a 69-year-old man who presented areas in which the hairs were cut short a few millimeters from the surface of the scalp. The examination of these hairs showed that their distal extremities presented a clean cut, or in other words, were severed with a sharp object.
Meiers,19 (1971) published a report of an identical case to that of Braun-Falco, and, in 1990, Orfanos and cols.20 reported another case similar to the others.
In all the described patients, the hair was cut almost at the surface of the scalp, with scissors or a razor,4 the hair thus being short and the distal extremities were cut off cleanly.
There is no trichotillomania in trichotemnomania, that is, the patient does not pull out the hair but cuts it off with some instrument. Obviously, the results of anatomicopathological exams, trichograms and capillary density will be within the normal limits. The diagnosis is made through dermatoscopy or with a simple optical microscope, which allows the observation of very short hairs that are cut off cleanly.
Unlike trichotillomania, trichotemnomania only occurs in senior adults, and all of the described cases were psychotic patients.
Runne,21 (2000) described a 70-year-old woman that presented three areas of alopecia on the scalp with diameters varying from 6 cm to 8 cm in the temporal and parietal regions, and on the nape of the neck. Under the dermatoscope some strands of brittle hair were observed. These gave the area a velvety aspect. In the periphery of the lesion the trichogram was normal, however the hair shafts in the center of the lesion presented trichoptilosis, trichorrexis nodosa and incomplete fractures and folds. Histopathology demonstrated intense acanthosis.
Every dermatologist is familiar with this situation. In any area of the body where there is frequent attrition, there may be breakage of the hairs and even alopecia. A typical example is the loss of the hair in the tibial area experienced by tailors, known as tailors' alopecia. This occurs because workers in this profession tend to cross their legs when they are sewing. It is also common to observe broken hair, giving the impression of having been shaved, in any pruriginous dermatosis, such as lichen planus, atopic dermatitis, plaque of neurodermatitis, etc. Obviously, any of these conditions may occur in the scalp.
Runne21 characterized the compulsive scratching of the scalp as scratching-pseudoalopecia. But in the opinion of the authors, this condition corresponds to the psychogenic excoriation described by Arnold and cols.,22 and Touraine.27
The term trichoteiromania was created by Freysschmidt and cols.,23 in 2001, to describe a syndrome in a 61-year-old woman who, over a period of several years, had presented areas in the scalp in which the hair was broken off at about two centimeters from the surface. The patient complained of intense itching in the area, which lead her to scratch a great deal.
Some hairs were collected with tweezers and when they were examined under a simple optical microscope, the hairs were observed to be about two centimeters in length with distal extremities split into a brush form. The surface of the skin of the scalp was erythematose and scaly as a consequence of the scratching.
Anatomicopathological exam revealed acanthosis, ortho-hyperkeratosis, focal parakeratosis and intraepidermic microvesiculation. The trichogram of these hairs was normal.
In the authors' opinion, the terms trichoteiromania and scratching-pseudoalopecia represent the same clinical entity. Both are caused by the action of attrition in the scalp and have as a common characteristic the presence of small hairs on the scalp with the distal extremities split into brush form. The skin reveals an epidermic thickening due to the attrition.
Sabouraud,24 (1913) described four cases of patients who presented hair shortened to a few centimeters from the scalp, he denominated this condition as trichosis of tonsure trichoclasia. Later, Jeanselme and col.,25 and Touraine and col.26 ddescribed cases similar to those of Sabouraud. Sanderson and col.,24 in 1970, published a report of eight cases that presented large areas where the hair was cut off only a few centimeters from the scalp. Sanderson called this condition tonsure trichotillomania. In none of these cases the authors described the characteristics of the remaining hairs, if they were anagenous or telogenous, nor the means by which they were cut off. Therefore, clinically they could correspond to trichotemnomania, trichoteiromania, trichocryptomania or trichorrexomania.
TRICHOCRYPTOMANIA OR TRICHORREXOMANIA
The terms trichocryptomania28 and trichorrexomania28 are synonymous and correspond to a compulsive action of cutting the hair with the nails. The patient may cut the hair at any level, close to the scalp or several centimeters from the surface of the skin. Usually an area of alopecia does not exist, but rather there are strands of hair cut in various lengths, giving a poor quality to the hairstyle. The hairs that suffer from the process of trichocryptomania or trichorrexomania, but are still attached to the scalp, present a brush appearance in their distal extremity.
Trichocryptomania may sometimes be part of the trichotillomania problem, in that, in addition to the patient pulling the hair, he may be breaking the hair with his nails.
In 1884, Le Page29 presented in the Department of Dermatology of the Royal Museum of the School of Surgeons of England, the case of a young woman, 17 years of age, with very entangled hair and which was starting to form into a hardened mass. Since the youth suffered from psychiatric problems, the author called the trichosis: "neuropathic plica". After that first description, other cases have been mentioned in the literature.30-33
Savil and col.34 uused the expression "neuropathic plica" when the matting of hair was accompanied by psychiatric problems. Neuropathic plica should be diagnosed differentially from acute compacting of the hair, an electrostatic phenomenon, that occurs in the hair during shampooing.35
The characterization of compulsive trichoses is not as simple as it seems. According to Toit and cols.,36 not all extraction of hair is compulsive; those authors called the patients that extract the hair as "hair-pullers", whether or not they presented trichotillomania, according to a psychiatric evaluation.
The management of a patient with compulsive trichosis is somewhat embarrassing for the dermatologist, especially if the patient and even the companion do not admit the possibility of self-aggression. A good knowledge of elementary trichology is fundamental for a precise diagnosis. As already seen, many times the manifestations are described from a clinical point of view, but without any emphasis on the characteristics of the hair. This hinders the understanding of the etiopathology of the disease.
There is a specific classification for each type of aggression to the hair. Patients have been observed, usually young with long hair, who simply cut their hair with their teeth, however without swallowing. The author believes that there is as yet no specific nomenclature for these cases and have denominated this condition: "trichodontoclasia."
Sometimes patients that are seriously psychotic may present profiles that are practically unclassifiable. There may be a combination of some or all of the aforementioned conditions, many times with even greater gravity. The patient, on some occasions, may violently pull out a large amount of hair at one time, or may even set his or her hair on fire.
It is considered that the dermatologist alone is insufficient to resolve the problem of a compulsive trichosis because the disease is linked to the symptomatology of a psychopathy, and thus, a professional in the field of psychology should also be consulted.
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Received on October 21, 2002.
Approved by the Consultive Council and accepted for publication on July 04, 2002.
* Work done at private clinic "Centro Dermatológico de Guarulhos"