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"The next thirteen cases (Nos. 12-24) were instances of anomalies of the mouth and nose. The 'bird's beak' (No. 12) may have been a markedly aquiline nose; No. 13 was a case of astoma; and Nos. 14 and 15 were instances of stenosis or atresia of the anterior nares. Fetuses with absence of the maxillae (Nos. 16 and 17) are in modern terminology called agnathous. Deformities like that existing in Nos. 20 and 21 have been observed in paracephalic and cyclopic fetuses. The coincident absence of nose and penis (No. 21) is interesting, especially when taken in conjunction with the popular belief that the size of the former organ varies with that of the latter. Enlargement of the upper lip (No. 22), called epimacrochelia by Taruffi, and absence of the lips (No. 23), known now under the name of brachychelia, have been not unfrequently noticed in recent times. The next six cases (Nos. 25-30) were instances of malformations of the upper limb: Nos. 25, 26. and 27 were probably instances of the so-called spontaneous or intrauterine amputation; and Nos. 28, 29, and 30 were examples of the comparatively common deformity known as polydactyly. No. 31 was probably a case of ectopia cordis.
"Then follow five instances of genital abnormalities (Nos. 32-36), consisting of absence of the penis (epispadias?), absence of penis and umbilicus (epispadias and exomphalos?), hermaphroditism, imperforate anus, and nondescent of one testicle. The nine following cases (Nos. 37-45) were anomalies of the lower limbs: Nos. 37, 38, and 42 may have been spontaneous amputations; Nos. 39 and 40 were doubtless instances of webbed toes (syndactyly), and the deformity indicated in No. 45 was presumably talipes equinus. The infant born with three feet (No. 43) was possibly a case of parasitic monstrosity, several of which have been reported in recent teratologic literature; but what is meant by the statement concerning 'male and female legs' it is not easy to determine.
"Certain of the ten following prodigies (Nos. 46-55) cannot in the present state of our knowledge be identified. The presence of congenital patches of white or gray hair on the scalp, as recorded in No. 46, is not an unknown occurrence at the present time; but what the Chaldeans meant by ipga, pinde, hali riksi, and kali on the head of the new-born infant it is impossible to tell. The guess may be hazarded that cephalhematoma, hydrocephalus, meningocele, nevi, or an excessive amount of vernix caseosa were the conditions indicated, but a wider acquaintance with the meaning of the cuneiform characters is necessary before any certain identification is possible. The 'pieces of skin hanging from the head' (No. 51) may have been fragments of the membranes; but there is nothing in the accompanying prediction to help us to trace the origin of the popular belief in the good luck following the baby born with a caul. If No. 53 was a case of congenital horns on the head, it must be regarded as a unique example, unless, indeed, a form of fetal ichthyosis be indicated.
"The remaining observations (No. 56-62) refer to cases of congenital teeth (No. 56) to deformity of the ears (Nos. 60 and 61), and a horn (No. 62)."
From these early times almost to the present day similar significance has been attached to minor structural anomalies. In the following pages the individual anomalies will be discussed separately and the most interesting examples of each will be cited. It is manifestly evident that the object of this chapter is to mention the most striking instances of abnormism and to give accompanying descriptions of associate points of interest, rather than to offer a scientific exposition of teratology, for which the reader is referred elsewhere.
Congenital defect of the epidermis and true skin is a rarity in pathology. Pastorello speaks of a child which lived for two and a half hours whose hands and feet were entirely destitute of epidermis; the true skin of those parts looked like that of a dead and already putrefying child. Hanks cites the history of a case of antepartum desquamation of the skin in a living fetus. Hochstetter describes a full-term, living male fetus with cutaneous defect on both sides of the abdomen a little above the umbilicus. The placenta and membranes were normal, a fact indicating that the defect was not due to amniotic adhesions; the child had a club-foot on the left side. The mother had a fall three weeks before labor.
Abnormal Elasticity of the Skin.—In some instances the skin is affixed so loosely to the underlying tissues and is possessed of so great elasticity that it can be stretched almost to the same extent as India rubber. There have been individuals who could take the skin of the forehead and pull it down over the nose, or raise the skin of the neck over the mouth. They also occasionally have an associate muscular development in the subcutaneous tissues similar to the panniculus adiposus of quadrupeds, giving them preternatural motile power over the skin. The man recently exhibited under the title of the "Elastic-Skin Man" was an example of this anomaly. The first of this class of exhibitionists was seen in Buda-Pesth some years since and possessed great elasticity in the skin of his whole body; even his nose could be stretched. Figure 70 represents a photograph of an exhibitionist named Felix Wehrle, who besides having the power to stretch his skin could readily bend his fingers backward and forward. The photograph was taken in January, 1888.
In these congenital cases there is loose attachment of the skin without hypertrophy, to which the term dermatolysis is restricted by Crocker. Job van Meekren, the celebrated Dutch physician of the seventeenth century, states that in 1657 a Spaniard, Georgius Albes, is reported to have been able to draw the skin of the left pectoral region to the left ear, or the skin under the face over the chin to the vertex. The skin over the knee could be extended half a yard, and when it retracted to its normal position it was not in folds. Seiffert examined a case of this nature in a young man of nineteen, and, contrary to Kopp's supposition, found that in some skin from over the left second rib the elastic fibers were quite normal, but there was transformation of the connective tissue of the dermis into an unformed tissue like a myxoma, with total disappearance of the connective-tissue bundles. Laxity of the skin after distention is often seen in multipara, both in the breasts and in the abdominal walls, and also from obesity, but in all such cases the skin falls in folds, and does not have a normal appearance like that of the true "elastic-skin man."
Occasionally abnormal development of the scalp is noticed. McDowall of twenty-two. On each side of the median line of the head there were five deep furrows, more curved and shorter as the distance from the median line increased. In the illustration the hair in the furrows is left longer than that on the rest of the head. The patient was distinctly microcephalic and the right side of the body was markedly wasted. The folds were due to hypertrophy of the muscles and scalp, and the same sort of furrowing is noticed when a dog "pricks his ears." This case may possibly be considered as an example of reversion to inferior types. Cowan records two cases of the foregoing nature in idiots. The first case was a paralytic idiot of thirty-nine, whose cranial development was small in proportion to the size of the face and body; the cranium was oxycephalic; the scalp was lax and redundant and the hair thin; there were 13 furrows, five on each side running anteroposteriorly, and three in the occipital region running transversely. The occipitofrontalis muscle had no action on them. The second case was that of an idiot of forty-four of a more degraded type than the previous one. The cranium was round and bullet-shaped and the hair generally thick. The scalp was not so lax as in the other case, but the furrows were more crooked. By tickling the scalp over the back of the neck the two median furrows involuntarily deepened.
Impervious Skin.—There have been individuals who claimed that their skin was impervious to ordinary puncture, and from time to time these individuals have appeared in some of the larger medical clinics of the world for inspection. According to a recent number of the London Graphic, there is in Berlin a Singhalese who baffles all investigations by physicians by the impenetrability of his skin. The bronzed Easterner, a Hercules in shape, claims to have found an elixir which will render the human skin impervious to any metal point or sharpened edge of a knife or dagger, and calls himself the "Man with Iron Skin." He is now exhibiting himself, and his greatest feat is to pass with his entire body through a hoop the inside of which is hardly big enough to admit his body and is closely set with sharp knife-points, daggers, nails, and similar things. Through this hoop he squeezes his body with absolute impunity. The physicians do not agree as to his immunity, and some of them think that Rhannin, which is his name, is a fakir who has by long practice succeeded in hardening himself against the impressions of metal upon his skin. The professors of the Berlin clinic, however, considered it worth while to lecture about the man's skin, pronouncing it an inexplicable matter. This individual performed at the London Alhambra in the latter part of 1895. Besides climbing with bare feet a ladder whose rungs were sharp-edged swords, and lying on a bed of nail points with four men seated upon him, he curled himself up in a barrel, through whose inner edges nails projected, and was rolled about the stage at a rapid rate. Emerging from thence uninjured, he gracefully bows himself off the stage.
Some individuals claim immunity from burns and show many interesting feats in handling fire. As they are nothing but skilful "fire jugglers" they deserve no mention here. The immunity of the participants in the savage fire ceremonies will be discussed in Chapter IX.
Albinism is characterized by the absolute or relative absence of pigment of the skin, due to an arrest, insufficiency, or retardation of this pigment. Following Trelat and Guinard, we may divide albinism into two classes,—general and partial.
As to the etiology of albinism, there is no known cause of the complete form. Heredity plays no part in the number of cases investigated by the authors. D'Aube, by his observations on white rabbits, believes that the influence of consanguinity is a marked factor in the production of albinism; there are, however, many instances of heredity in this anomaly on record, and this idea is possibly in harmony with the majority of observers. Geoffroy-Saint-Hilaire has noted that albinism can also be a consequence of a pathologic condition having its origin in adverse surroundings, the circumstances of the parents, such as the want of exercise, nourishment, light, etc.
Lesser knew a family in which six out of seven were albinos, and in some tropical countries, such as Loango, Lower Guinea, it is said to be endemic. It is exceptional for the parents to be affected; but in a case of Schlegel, quoted by Crocker, the grandfather was an albino, and Marey describes the case of the Cape May albinos, in which the mother and father were "fair emblems of the African race," and of their children three were black and three were white, born in the following order: two consecutive black boys, two consecutive white girls, one black girl, one white boy. Sym of Edinburgh relates the history of a family of seven children, who were alternately white and black. All but the seventh were living and in good health and mentally without defect. The parents and other relatives were dark. Figure 73 portrays an albino family by the name of Cavalier who exhibited in Minneapolis in 1887.
Examples of the total absence of pigment occur in all races, but particularly is it interesting when seen in negroes who are found absolutely white but preserving all the characteristics of their race, as, for instance, the kinky, woolly hair, flattened nose, thick lips, etc. Rene Claille, in his "Voyage a Tombouctou," says that he saw a white infant, the offspring of a negro and negress. Its hair was white, its eyes blue, and its lashes flaxen. Its pupils were of a reddish color, and its physiognomy that of a Mandingo. He says such cases are not at all uncommon; they are really negro albinos. Thomas Jefferson, in his "History of Virginia," has an excellent description of these negroes, with their tremulous and weak eyes; he remarks that they freckle easily. Buffon speaks of Ethiops with white twins, and says that albinos are quite common in Africa, being generally of delicate constitution, twinkling eyes, and of a low degree of intelligence; they are despised and ill-treated by the other negroes. Prichard, quoted by Sedgwick, speaks of a case of atavic transmission of albinism through the male line of the negro race. The grandfather and the grandchild were albinos, the father being black. There is a case of a brother and sister who were albinos, the parents being of ordinary color but the grandfather an albino. Coinde, quoted by Sedgwick, speaks of a man who, by two different wives, had three albino children.
A description of the ordinary type of albino would be as follows: The skin and hair are deprived of pigment; the eyebrows and eyelashes are of a brilliant white or are yellowish; the iris and the choroid are nearly or entirely deprived of coloring material, and in looking at the eye we see a roseate zone and the ordinary pink pupil; from absence of pigment they necessarily keep their eyes three-quarters closed, being photophobic to a high degree. They are amblyopic, and this is due partially to a high degree of ametropia (caused by crushing of the eyeball in the endeavor to shut out light) and from retinal exhaustion and nystagmus. Many authors have claimed that they have little intelligence, but this opinion is not true. Ordinarily the reproductive functions are normal, and if we exclude the results of the union of two albinos we may say that these individuals are fecund.
Partial albinism is seen. The parts most often affected are the genitals, the hair, the face, the top of the trunk, the nipple, the back of the hands and fingers. Folker reports the history of a case of an albino girl having pink eyes and red hair, the rest of the family having pink eyes and white hair. Partial albinism, necessarily congenital, presenting a piebald appearance, must not be confounded with leukoderma, which is rarely seen in the young and which will be described later.
Albinism is found in the lower animals, and is exemplified ordinarily by rats, mice, crows, robins, etc. In the Zoologic Garden at Baltimore two years ago was a pair of pure albino opossums. The white elephant is celebrated in the religious history of Oriental nations, and is an object of veneration and worship in Siam. White monkeys and white roosters are also worshiped. In the Natural History Museum in London there are stuffed examples of albinism and melanism in the lower animals.
Melanism is an anomaly, the exact contrary of the preceding. It is characterized by the presence in the tissues and skin of an excessive amount of pigment. True total melanism is unknown in man, in whom is only observed partial melanism, characterized simply by a pronounced coloration of part of the integument.
Some curious instances have been related of an infant with a two-colored face, and of others with one side of the face white and the other black; whether they were cases of partial albinism or partial melanism cannot be ascertained from the descriptions.
Such epidermic anomalies as ichthyosis, scleroderma, and molluscum simplex, sometimes appearing shortly after birth, but generally seen later in life, will be spoken of in the chapter on Anomalous Skin Diseases.
Human horns are anomalous outgrowths from the skin and are far more frequent than ordinarily supposed. Nearly all the older writers cite examples. Aldrovandus, Amatus Lusitanus, Boerhaave, Dupre, Schenck, Riverius, Vallisneri, and many others mention horns on the head. In the ancient times horns were symbolic of wisdom and power. Michael Angelo in his famous sculpture of Moses has given the patriarch a pair of horns. Rhodius observed a Benedictine monk who had a pair of horns and who was addicted to rumination. Fabricius saw a man with horns on his head, whose son ruminated; the son considered that by virtue of his ruminating characteristics his father had transmitted to him the peculiar anomaly of the family. Fabricius Hildanus saw a patient with horns all over the body and another with horns on the forehead. Gastaher speaks of a horn from the left temple; Zacutus Lusitanus saw a horn from the heel; Wroe, one of considerable length from the scapula; Cosnard, one from the bregma; the Ephemerides, from the foot; Borellus, from the face and foot, and Ash, horns all over the body. Home, Cooper, and Treves have collected examples of horns, and there is one 11 inches long and 2 1/2 in circumference in a London museum. Lozes collected reports of 71 cases of horns,—37 in females, 31 in males, and three in infants. Of this number, 15 were on the head, eight on the face, 18 on the lower extremities, eight on the trunk, and three on the glans penis. Wilson collected reports of 90 cases,—44 females, 39 males, the sex not being mentioned in the remainder. Of these 48 were on the head, four on the face, four on the nose, 11 on the thigh, three on the leg and foot, six on the back, five on the glans penis, and nine on the trunk. Lebert's collection numbered 109 cases of cutaneous horns. The greater frequency among females is admitted by all authors. Old age is a predisposing cause. Several patients over seventy have been seen and one of ninety-seven.
Instances of cutaneous horns, when seen and reported by the laity, give rise to most amusing exaggerations and descriptions. The following account is given in New South Wales, obviously embellished with apocryphal details by some facetious journalist: The child, five weeks old, was born with hair two inches long all over the body; his features were fiendish and his eyes shone like beads beneath his shaggy brows. He had a tail 18 inches long, horns from the skull, a full set of teeth, and claw-like hands; he snapped like a dog and crawled on all fours, and refused the natural sustenance of a normal child. The mother almost became an imbecile after the birth of the monster. The country people about Bomballa considered this devil-child a punishment for a rebuff that the mother gave to a Jewish peddler selling Crucifixion-pictures. Vexed by his persistence, she said she would sooner have a devil in her house than his picture.
Lamprey has made a minute examination of the much-spoken-of "Horned Men of Africa." He found that this anomaly was caused by a congenital malformation and remarkable development of the infraorbital ridge of the maxillary bone. He described several cases, and through an interpreter found that they were congenital, followed no history of traumatism, caused little inconvenience, and were unassociated with disturbance of the sense of smell. He also learned that the deformity was quite rare in the Cape Coast region, and received no information tending to prove the conjecture that the tribes in West Africa used artificial means to produce the anomaly, although such custom is prevalent among many aborigines.
Probably the most remarkable case of a horn was that of Paul Rodrigues, a Mexican porter, who, from the upper and lateral part of his head, had a horn 14 inches in circumference and divided into three shafts, which he concealed by constantly wearing a peculiarly shaped red cap. There is in Paris a wax model of a horn, eight or nine inches in length, removed from an old woman by the celebrated Souberbielle. Figure 75 is from a wax model supposed to have been taken from life, showing an enormous grayish-black horn proceeding from the forehead. Warren mentions a case under the care of Dubois, in a woman from whose forehead grew a horn six inches in diameter and six inches in height. It was hard at the summit and had a fetid odor. In 1696 there was an old woman in France who constantly shed long horns from her forehead, one of which was presented to the King. Bartholinus mentions a horn 12 inches long. Voigte cites the case of an old woman who had a horn branching into three portions, coming from her forehead. Sands speaks of a woman who had a horn 6 3/4 inches long, growing from her head. There is an account of the extirpation of a horn nearly ten inches in length from the forehead of a woman of eighty-two. Bejau describes a woman of forty from whom he excised an excrescence resembling a ram's horn, growing from the left parietal region. It curved forward and nearly reached the corresponding tuberosity. It was eight cm. long, two cm. broad at the base, and 1 1/2 cm. at the apex, and was quite mobile. It began to grow at the age of eleven and had constantly increased. Vidal presented before the Academie de Medecine in 1886 a twisted horn from the head of a woman. This excrescence was ten inches long, and at the time of presentation reproduction of it was taking place in the woman. Figure 76 shows a case of ichthyosis cornea pictured in the Lancet, 1850.
There was a woman of seventy-five, living near York, who had a horny growth from the face which she broke off and which began to reproduce, the illustration representing the growth during twelve months. Lall mentions a horn from the cheek; Gregory reports one that measured 7 1/2 inches long that was removed from the temple of a woman in Edinburgh; Chariere of Barnstaple saw a horn that measured seven inches growing from the nape of a woman's neck; Kameya Iwa speaks of a dermal horn of the auricle; Saxton of New York has excised several horns from the tympanic membrane of the ear; Noyes speaks of one from the eyelid; Bigelow mentions one from the chin; Minot speaks of a horn from the lower lip, and Doran of one from the neck.
Gould cites the instance of a horn growing from an epitheliomatous penis. The patient was fifty-two years of age and the victim of congenital phimosis. He was circumcised four years previously, and shortly after the wound healed there appeared a small wart, followed by a horn about the size of a marble. Jewett speaks of a penile horn 3 1/2 inches long and 3 3/4 inches in diameter; Pick mentions one 2 1/2 inches long. There is an account of a Russian peasant boy who had a horn on his penis from his earliest childhood. Johnson mentions a case of a horn from the scrotum, which was of sebaceous origin and was subsequently supplanted by an epithelioma.
Ash reported the case of a girl named Annie Jackson, living in Waterford, Ireland, who had horny excrescences from her joints, arms, axillae, nipples, ears, and forehead. Locke speaks of a boy at the Hopital de la Charite in Paris, who had horny excrescences four inches long and 11 inches in circumference growing from his fingers and toes.
Wagstaffe presents a horn which grew from the middle of the leg six inches below the knee in a woman of eighty. It was a flattened spiral of more than two turns, and during forty years' growth had reached the length of 14.3 inches. Its height was 3.8 inches, its skin-attachment 1.5 inches in diameter, and it ended in a blunt extremity of 0.5 inch in diameter. Stephens mentions a dermal horn on the buttocks at the seat of a carcinomatous cicatrix. Harris and Domonceau speak of horns from the leg. Cruveilhier saw a Mexican Indian who had a horn four inches long and eight inches in circumference growing from the left lumbar region. It had been sawed off twice by the patient's son and was finally extirpated by Faget. The length of the pieces was 12 inches. Bellamy saw a horn on the clitoris about the size of a tiger's claw in a its origin from beneath the preputium clitoridis.
Horns are generally solitary but cases of multiple formation are known Lewin and Heller record a syphilitic case with eight cutaneous horns on the palms and soles. A female patient of Manzuroff had as many as 185 horns.
Pancoast reports the case of a man whose nose, cheeks, forehead, and lips were covered with horny growths, which had apparently undergone epitheliomatous degeneration. The patient was a sea-captain of seventy-eight, and had been exposed to the winds all his life. He had suffered three attacks of erysipelas from prolonged exposure. When he consulted Pancoast the horns had nearly all fallen off and were brought to the physician for inspection; and the photograph was taken after the patient had tied the horns in situ on his face.
Anomalies of the Hair.—Congenital alopecia is quite rare, and it is seldom that we see instances of individuals who have been totally destitute of hair from birth. Danz knew of two adult sons of a Jewish family who never had hair or teeth. Sedgwick quotes the case of a man of fifty-eight who ever since birth was totally devoid of hair and in whom sensible perspiration and tears were absent. A cousin on his mother's side, born a year before him, had precisely the same peculiarity. Buffon says that the Turks and some other people practised depilatory customs by the aid of ointments and pomades, principally about the genitals. Atkinson exhibited in Philadelphia a man of forty who never had any distinct growth of hair since birth, was edentulous, and destitute of the sense of smell and almost of that of taste. He had no apparent perspiration, and when working actively he was obliged to wet his clothes in order to moderate the heat of his body. He could sleep in wet clothes in a damp cellar without catching cold. There was some hair in the axillae and on the pubes, but only the slightest down on the scalp, and even that was absent on the skin. His maternal grandmother and uncle were similarly affected; he was the youngest of 21 children, had never been sick, and though not able to chew food in the ordinary manner, he had never suffered from dyspepsia in any form. He was married and had eight children. Of these, two girls lacked a number of teeth, but had the ordinary quantity of hair. Hill speaks of an aboriginal man in Queensland who was entirely devoid of hair on the head, face, and every part of the body. He had a sister, since dead, who was similarly hairless. Hill mentions the accounts given of another black tribe, about 500 miles west of Brisbane, that contained hairless members. This is very strange, as the Australian aboriginals are a very hairy race of people.
Hutchinson mentions a boy of three and a half in whom there was congenital absence of hair and an atrophic condition of the skin and appendages. His mother was bald from the age of six, after alopecia areata. Schede reports two cases of congenitally bald children of a peasant woman (a boy of thirteen and a girl of six months). They had both been born quite bald, and had remained so. In addition there were neither eyebrows nor eyelashes and nowhere a trace of lanugo. The children were otherwise healthy and well formed. The parents and brothers were healthy and possessed a full growth of hair. Thurman reports a case of a man of fifty-eight, who was almost devoid of hair all his life and possessed only four teeth. His skin was very delicate and there was absence of sensible perspiration and tears. The skin was peculiar in thinness, softness, and absence of pigmentation. The hair on the crown of the head and back was very fine, short, and soft, and not more in quantity than that of an infant of three months. There was a similar peculiarity in his cousin-german. Williams mentions the case of a young lady of fifteen with scarcely any hair on the eyebrows or head and no eyelashes. She was edentulous and had never sensibly perspired. She improved under tonic treatment.
Rayer quotes the case of Beauvais, who was a patient in the Hopital de la Charite in 1827. The skin of this man's cranium was apparently completely naked, although in examining it narrowly it was found to be beset with a quantity of very white and silky hair, similar to the down that covers the scalp of infants; here and there on the temples there were a few black specks, occasioned by the stumps of several hairs which the patient had shaved off. The eyebrows were merely indicated by a few fine and very short hairs; the free edges of the eyelids were without cilia, but the bulb of each of these was indicated by a small, whitish point. The beard was so thin and weak that Beauvais clipped it off only every three weeks. A few straggling hairs were observed on the breast and pubic region, as in young people on the approach of puberty. There was scarcely any under the axillae. It was rather more abundant on the inner parts of the legs. The voice was like that of a full-grown and well-constituted man. Beauvais was of an amorous disposition and had had syphilis twice. His mother and both sisters had good heads of hair, but his father presented the same defects as Beauvais.
Instances are on record of women devoid of hair about the genital region. Riolan says that he examined the body of a female libertine who was totally hairless from the umbilical region down.
Congenital alopecia is seen in animals. There is a species of dog, a native of China but now bred in Mexico and in the United States, which is distinguished for its congenital alopecia. The same fact has been observed occasionally in horses, cattle, and dogs. Heusner has seen a pigeon destitute of feathers, and which engendered a female which in her turn transmitted the same characteristic to two of her young.
Sexualism and Hair Growth.—The growth or development of the hair may be accelerated by the state of the organs of generation. This is peculiarly noticeable in the pubic hairs and the beard, and is fully exemplified in the section on precocious development (Chapter VII); however, Moreau de la Sarthe showed a child to the Medical Faculty of Paris in whom precocious development of the testicles had influenced that of the hair to such a degree that, at the age of six, the chest of this boy was as thickly set with hair as is usually seen in adults. It is well known that eunuchs often lose a great part of their beards, and after removal of the ovaries women are seen to develop an extra quantity of hair. Gerberon tells of an infant with a beard, and Paullini and the Ephemerides mention similar instances.
Bearded women are not at all infrequent. Hippocrates mentions a female who grew a beard shortly after menstruation had ceased. It is a well-recognized fact that after the menopause women become more hirsute, the same being the case after removal of any of the functional generative apparatus. Vicat saw a virgin who had a beard, and Joch speaks of "foeminis barbati." Leblond says that certain women of Ethiopia and South America have beards and little or no menstruation. He also says that sterility and excessive chastity are causes of female beards, and cites the case of Schott of a young widow who secluded herself in a cloister, and soon had a beard.
Barbara Urster, who lived in the 16th century, had a beard to her girdle. The most celebrated "bearded woman" was Rosine-Marguerite Muller, who died in a hospital in Dresden in 1732, with a thick beard and heavy mustache. Julia Pastrana had her face covered with thick hair and had a full beard and mustache. She exhibited defective dentition in both jaws, and the teeth present were arranged in an irregular fashion. She had pronounced prognathism, which gave her a simian appearance. Ecker examined in 1876 a woman who died at Fribourg, whose face contained a full beard and a luxuriant mustache.
Harris reports several cases of bearded women, inmates of the Coton Hill Lunatic Asylum. One of the patients was eighty-three years of age and had been insane forty-four years following a puerperal period. She would not permit the hair on her face to be cut, and the curly white hairs had attained a length of from eight to ten inches on the chin, while on the upper lip the hairs were scarcely an inch. This patient was quite womanly in all her sentiments. The second case was a woman of thirty-six, insane from emotional melancholia. She had tufts of thick, curly hair on the chin two inches long, light yellowish in color, and a few straggling hairs on the upper lip. The third case was that of a woman of sixty-four, who exhibited a strong passion for the male sex. Her menstruation had been regular until the menopause. She plaited her beard, and it was seven or eight inches long on the chin and one inch on the lip. This woman had extremely hairy legs. Another case was that of a woman of sixty-two, who, though bald, developed a beard before the climacteric. Her structural proportions were feminine in character, and it is said that her mother, who was sane, had a beard also. A curious case was that of a woman of twenty-three (Mrs. Viola M.), who from the age of three had a considerable quantity of hair on the side of the cheek which eventually became a full beard. She was quite feminine was free from excessive hair elsewhere, her nose and forehead being singularly bare. Her voice was very sweet; she was married at seventeen and a half, having two normal children, and nursed each for one month. "The bearded woman" of every circus side-show is an evidence of the curious interest in which these women are held. The accompanying illustration is a representation of a "bearded woman" born in Bracken County, Ky. Her beard measured 15 inches in length.
There is a class of anomalies in which there is an exaggerated development of hair. We would naturally expect to find the primitive peoples, who are not provided with artificial protection against the wind, supplied with an extra quantity of hair or having a hairy coat like animals; but this is sometimes found among civilized people. This abnormal presence of hair on the human body has been known for many years; the description of Esau in the Bible is an early instance. Aldrovandus says that in the sixteenth century there came to the Canary Islands a family consisting of a father, son, and two daughters, who were covered all over their bodies by long hair, and their portrait, certainly reproduced from life, resembles the modern instances of "dog men."
In 1883 there was shown in England and France, afterward in America, a girl of seven named "Krao," a native of Indo-China. The whole body of this child was covered with black hair. Her face was of the prognathic type, and this, with her extraordinary prehensile powers of feet and lips, gave her the title of "Darwin's missing link." In 1875 there was exhibited in Paris, under the name of "l'homme-chien" Adrien Jeftichew, a Russian peasant of fifty-five, whose face, head, back, and limbs were covered with a brown hairy coat looking like wool and several centimeters long. The other parts of the body were also covered with hair, but less abundantly. This individual had a son of three, Theodore, who was hairy like himself.
A family living in Burmah (Shive-Maon, whose history is told by Crawford and Yule), consisting of a father, a daughter, and a granddaughter, were nearly covered with hair. Figure 84 represents a somewhat similar family who were exhibited in this country.
Teresa Gambardella, a young girl of twelve, mentioned by Lombroso, was covered all over the body, with the exception of the hands and feet, by thick, bushy hair. This hypertrichosis was exemplified in this country only a few months since by a person who went the rounds of the dime museums under the euphonious name of "Jo-Jo, the dog-face boy." His face was truly that of a skye-terrier.
Sometimes the hairy anomalies are but instances of naevus pilosus. The Indian ourang-outang woman examined at the office of the Lancet was an example of this kind. Hebra, Hildebrandt, Jablokoff, and Klein describe similar cases. Many of the older "wild men" were individuals bearing extensive hairy moles.
Rayer remarks that he has seen a young man of sixteen who exhibited himself to the public under the name of a new species of wild man whose breast and back were covered with light brown hair of considerable length.
The surface upon which it grew was of a brownish hue, different from the color of the surrounding integument. Almost the whole of the right arm was covered in the same manner. On the lower extremity several tufts of hair were observed implanted upon brown spots from seven to eight lines in diameter symmetrically disposed upon both legs. The hair was brown, of the same color as that of the head. Bichat informs us that he saw at Paris an unfortunate man who from his birth was afflicted with a hairy covering of his face like that of a wild boar, and he adds that the stories which were current among the vulgar of individuals with a boar's head, wolf's head, etc., undoubtedly referred to cases in which the face was covered to a greater or less degree with hair. Villerme saw a child of six at Poitiers in 1808 whose body, except the feet and hands, was covered with a great number of prominent brown spots of different dimensions, beset with hair shorter and not so strong as that of a boar, but bearing a certain resemblance to the bristles of that animal. These spots occupied about one-fifth of the surface of this child's skin. Campaignac in the early part of this century exhibited a case in which there was a large tuft of long black hair growing from the shoulder. Dufour has detailed a case of a young man of twenty whose sacral region contained a tuft of hair as long and black, thick and pliant, as that of the head, and, particularly remarkable in this case, the skin from which it grew was as fine and white as the integument of the rest of the body. There was a woman exhibited recently, under the advertisement of "the lady with a mane," who had growing from the center of her back between the shoulders a veritable mane of long, black hair, which doubtless proceeded from a form of naevus.
Duyse reports a case of extensive hypertrichosis of the back in a girl aged nine years; her teeth were normal; there was pigmentation of the back and numerous pigmentary nevi on the face. Below each scapula there were tumors of the nature of fibroma molluscum. In addition to hairy nevi on the other parts of the body there was localized ichthyosis.
Ziemssen figures an interesting case of naevus pilosus resembling "bathing tights". There were also present several benign tumors (fibroma molluscum) and numerous smaller nevi over the body. Schulz first observed the patient in 1878. This individual's name was Blake, and he stated that he was born with a large naevus spreading over the upper parts of the thighs and lower parts of the trunk, like bathing-tights, and resembling the pelt of an animal. The same was true of the small hairy parts and the larger and smaller tumors. Subsequently the altered portions of the skin had gradually become somewhat larger. The skin of the large hairy naevus, as well as that of the smaller ones, was stated by Schulz to have been in the main thickened, in part uneven, verrucose, from very light to intensely dark brown in color; the consistency of the larger mammiform and smaller tumors soft, doughy, and elastic. The case was really one of large congenital naevus pilosus and fibroma molluscum combined.
A Peruvian boy was shown at the Westminster Aquarium with a dark, hairy mole situated in the lower part of the trunk and on the thighs in the position of bathing tights. Nevins Hyde records two similar cases with dermatolytic growths. A sister of the Peruvian boy referred to had a still larger growth, extending from the nucha all over the back. Both she and her brother had hundreds of smaller hairy growths of all sizes scattered irregularly over the face, trunk, and limbs. According to Crocker, a still more extraordinary case, with extensive dermatolytic growths all over the back and nevi of all sizes elsewhere, is described and engraved in "Lavater's Physiognomy," 1848. Baker describes an operation in which a large mole occupying half the forehead was removed by the knife.
In some instances the hair and beard is of an enormous length. Erasmus Wilson of London saw a female of thirty-eight, whose hair measured 1.65 meters long. Leonard of Philadelphia speaks of a man in the interior of this country whose beard trailed on the ground when he stood upright, and measured 2.24 meters long. Not long ago there appeared the famous so-called "Seven Sutherland Sisters," whose hair touched the ground, and with whom nearly every one is familiar through a hair tonic which they extensively advertised. In Nature, January 9, 1892, is an account of a Percheron horse whose mane measured 13 feet and whose tail measured almost ten feet, probably the greatest example of excessive mane development on record. Figure 88 represents Miss Owens, an exhibitionist, whose hair measured eight feet three inches. In Leslie's Weekly, January 2, 1896, there is a portrait of an old negress named Nancy Garrison whose woolly hair was equally as long.
The Ephemerides contains the account of a woman who had hair from the mons veneris which hung to the knees; it was affected with plica polonica, as was also the other hair of the body.
Rayer saw a Piedmontese of twenty-eight, with an athletic build, who had but little beard or hair on the trunk, but whose scalp was covered with a most extraordinary crop. It was extremely fine and silky, was artificially frizzled, dark brown in color, and formed a mass nearly five feet in circumference.
Certain pathologic conditions may give rise to accidental growths of hair. Boyer was accustomed to quote in his lectures the case of a man who, having an inflamed tumor in the thigh, perceived this part becoming covered in a short time with numerous long hairs. Rayer speaks of several instances of this kind. In one the part affected by a blister in a child of two became covered with hair. Another instance was that of a student of medicine, who after bathing in the sea for a length of time, and exposing himself to the hot sun, became affected with coppery patches, from which there sprang a growth of hair. Bricheteau, quoted by the same authority, speaks of a woman of twenty-four, having white skin and hair of deep black, who after a long illness occasioned by an affection analogous to marasmus became covered, especially on the back, breast, and abdomen, with a multitude of small elevations similar to those which appear on exposure to cold. These little elevations became brownish at the end of a few days, and short, fair, silky hair was observed on the summit of each, which grew so rapidly that the whole surface of the body with the exception of the hands and face became velvety. The hair thus evolved was afterward thrown out spontaneously and was not afterward reproduced.
Anomalies of the Color of the Hair.—New-born infants sometimes have tufts of hair on their heads which are perfectly white in color. Schenck speaks of a young man whose beard from its first appearance grew white. Young men from eighteen to twenty occasionally become gray; and according to Rayer, paroxysms of rage, unexpected and unwelcome news, diseases of the scalp such as favus, wounds of the head, habitual headache, over-indulgence of the sexual appetite, mercurial courses too frequently repeated, too great anxiety, etc., have been known to blanch the hair prematurely.
The well-accepted fact of the sudden changing of the color of the hair from violent emotions or other causes has always excited great interest, and many ingenious explanations have been devised to account for it. There is a record in the time of Charles V of a young man who was committed to prison in 1546 for seducing his girl companion, and while there was in great fear and grief, expecting a death-sentence from the Emperor the next day. When brought before his judge, his face was wan and pale and his hair and beard gray, the change having taken place in the night. His beard was filthy with drivel, and the Emperor, moved by his pitiful condition, pardoned him. There was a clergyman of Nottingham whose daughter at the age of thirteen experienced a change from jet-blackness of the hair to white in a single night, but this was confined to a spot on the back of the head 1 1/2 inches in length. Her hair soon became striped, and in seven years was totally white. The same article speaks of a girl in Bedfordshire, Maria Seeley, aged eight, whose face was swarthy, and whose hair was long and dark on one side and light and short on the other. One side of her body was also brown, while the other side was light and fair. She was seen by the faculty in London, but no cause could be established.
Voigtel mentions the occurrence of canities almost suddenly. Bichat had a personal acquaintance whose hair became almost entirely gray in consequence of some distressing news that reached him. Cassan records a similar case. According to Rayer, a woman by the name of Perat, summoned before the Chamber of Peers to give evidence in the trial of the assassin Louvel, was so much affected that her hair became entirely white in a single night Byron makes mention of this peculiar anomaly in the opening stanzas of the "Prisoner of Chillon:"—
"My hair is gray, but not with years, Nor grew it white In a single night. As men's have grown from sudden fears."
The commentators say that Byron had reference to Ludovico Sforza and others. The fact of the change is asserted of Marie Antoinette, the wife of Louis XVI, though in not quite so short a period, grief and not fear being the cause. Ziemssen cites Landois' case of a compositor of thirty-four who was admitted to a hospital July 9th with symptoms of delirium tremens; until improvement began to set in (July 13th) he was continually tormented by terrifying pictures of the imagination. In the night preceding the day last mentioned the hair of the head and beard of the patient, formerly blond, became gray. Accurate examination by Landois showed the pigment contents of the hair to be unchanged, and led him to believe that the white color was solely due to the excessive development of air-bubbles in the hair shaft. Popular belief brings the premature and especially the sudden whitening into connection with depressing mental emotions. We might quote the German expression—"Sich graue Haare etwas wachsen lassen" ("To worry one's self gray"). Brown-Sequard observed on several occasions in his own dark beard hairs which had turned white in a night and which he epileptoid. He closes his brief communication on the subject with the belief that it is quite possible for black hair to turn white in one night or even in a less time, although Hebra and Kaposi discredit sudden canities (Duhring). Raymond and Vulpian observed a lady of neurotic type whose hair during a severe paroxysm of neuralgia following a mental strain changed color in five hours over the entire scalp except on the back and sides; most of the hair changed from black to red, but some to quite white, and in two days all the red hair became white and a quantity fell off. The patient recovered her general health, but with almost total loss of hair, only a few red, white, and black hairs remaining on the occipital and temporal regions. Crocker cites the case of a Spanish cock which was nearly killed by some pigs. The morning after the adventure the feathers of the head had become completely white, and about half of those on the back of the neck were also changed.
Dewees reports a case of puerperal convulsions in a patient under his care which was attended with sudden canities. From 10 A.M. to 4 P.M. 50 ounces of blood were taken. Between the time of Dr. Dewees' visits, not more than an hour, the hair anterior to the coronal suture turned white. The next day it was less light, and in four or five days was nearly its natural color. He also mentions two cases of sudden blanching from fright.
Fowler mentions the case of a healthy girl of sixteen who found one morning while combing her hair, which was black, that a strip the whole length of the back hair was white, starting from a surface about two inches square around the occipital protuberance. Two weeks later she had patches of ephelis over the whole body.
Prentiss, in Science, October 3, 1890, has collected numerous instances of sudden canities, several of which will be given:—
"In the Canada Journal of Medical Science, 1882, p. 113, is reported a case of sudden canities due to business-worry. The microscope showed a great many air-vesicles both in the medullary substance and between the medullary and cortical substance.
"In the Boston Medical and Surgical Journal, 1851, is reported a case of a man thirty years old, whose hair 'was scared' white in a day by a grizzly bear. He was sick in a mining camp, was left alone, and fell asleep. On waking he found a grizzly bear standing over him.
"A second case is that of a man of twenty-three years who was gambling in California. He placed his entire savings of $1100 on the turn of a card. He was under tremendous nervous excitement while the cards were being dealt. The next day his hair was perfectly white.
"In the same article is the statement that the jet-black hair of the Pacific Islanders does not turn gray gradually, but when it does turn it is sudden, usually the result of fright or sudden emotions."
D'Alben, quoted by Fournier, describes a young man of twenty-four, an officer in the regiment of Touraine in 1781, who spent the night in carnal dissipation with a mulatto, after which he had violent spasms, rendering flexion of the body impossible. His beard and hair on the right side of the body was found as white as snow, the left side being unchanged. He appeared before the Faculte de Montpelier, and though cured of his nervous symptoms his hair was still white, and no suggestion of relief was offered him.
Louis of Bavaria, who died in 1294, on learning of the innocence of his wife, whom he had put to death on a suspicion of her infidelity, had a change of color in his hair, which became white almost immediately. Vauvilliers, the celebrated Hellenist, became white-haired almost immediately after a terrible dream, and Brizard, the comedian, experienced the same change after a narrow escape from drowning in the Rhone. The beard and the hair of the Duke of Brunswick whitened in twenty-four hours after hearing that his father had been mortally wounded at the battle of Auerstadt.
De Schweinitz speaks of a well-formed and healthy brunette of eighteen in whom the middle portion of the cilia of the right upper eyelid and a number of the hairs of the lower lid turned white in a week. Both eyes were myopic, but no other cause could be assigned. Another similar case is cited by Hirshberg, and the authors have seen similar cases. Thornton of Margate records the case of a lady in whom the hair of the left eyebrow and eyelashes began to turn white after a fortnight of sudden grief, and within a week all the hair of these regions was quite white and remained so. No other part was affected nor was there any other symptom. After a traumatic ophthalmitis of the left and sympathetic inflammation of the right eye in a boy of nine, Schenck observed that a group of cilia of the right upper lid and nearly all the lashes of the upper lid of the left eye, which had been enucleated, turned silvery-white in a short time. Ludwig has known the eyelashes to become white after small-pox. Communications are also on record of local decolorization of the eyebrows and lashes in neuralgias of isolated branches of the trigeminus, especially of the supraorbital nerve.
Temporary and Partial Canities.—Of special interest are those cases in which whiteness of the hair is only temporary. Thus, Compagne mentions a case in which the black hair of a woman of thirty-six began to fade on the twenty-third day of a malignant fever, and on the sixth day following was perfectly white, but on the seventh day the hairs became darker again, and on the fourteenth day after the change they had become as black as they were originally. Wilson records a case in which the hair lost its color in winter and regained it in summer. Sir John Forbes, according to Crocker, had gray hair for a long time, then suddenly it all turned white, and after remaining so for a year it returned to its original gray.
Grayness of the hair is sometimes only partial. According to Crocker an adult whose hair was generally brown had a tuft of white hair over the temple, and several like cases are on record. Lorry tells us that grayness of one side only is sometimes occasioned by severe headache. Hagedorn has known the beard to be black in one place and white in another. Brandis mentions the hair becoming white on one side of the face while it continued of its former color on the other. Rayer quotes cases of canities of the whole of one side of the body.
Richelot observed white mottling of hair in a girl sick with chlorosis. The whitening extended from the roots to a distance of two inches. The probable cause was a temporary alteration of the pigment-forming function. When the chlorosis was cured the natural color returned. Paullini and Riedlin, as well as the Ephemerides, speak of different colored hair in the same head, and it is not at all rare to see individuals with an anomalously colored patch of hair on the head. The members of the ancient house of Rohan were said to possess a tuft of white hair on the front of their heads.
Michelson of Konigsberg describes a curious case in a barrister of twenty-three affected with partial canities. In the family of both parents there was stated to be congenital premature canities, and some white hairs had been observed even in childhood. In the fifteenth year, after a grave attack of scarlet fever, the hair to a great extent fell out. The succeeding growth of hair was stated to have been throughout lighter in tissue and color and fissured at the points. Soon after bunches of white hair appeared on the occiput, and in the succeeding years small patches of decolored hairs were observed also on the anterior and lateral portions of the scalp. In the spring of 1880 the patient exhibited signs of infiltration of the apex of the right lung, and afterward a violent headache came on. At the time of the report the patient presented the appearance shown in Figure 89. The complexion was delicate throughout, the eyelashes and eyelids dark brown, the moustache and whiskers blond, and in the latter were a few groups of white hair. The white patches were chiefly on the left side of the head. The hairs growing on them were unpigmented, but otherwise normal. The patient stated that his head never sweated. He was stout and exhibited no signs of internal disease, except at the apex of the right lung.
Anomalous Color Changes of the Hair.—The hair is liable to undergo certain changes of color connected with some modification of that part of the bulb secreting its coloring-matter. Alibert, quoted by Rayer, gives us a report of the case of a young lady who, after a severe fever which followed a very difficult labor, lost a fine head of hair during a discharge of viscid fluid, which inundated the head in every part. He tells us, further, that the hair grew again of a deep black color after the recovery of the patient. The same writer tells of the case of James B—, born with brown hair, who, having lost it all during the course of a sickness, had it replaced with a crop of the brightest red. White and gray hair has also, under peculiar circumstances, been replaced by hair of the same color as the individual had in youth. We are even assured by Bruley that in 1798 the white hair of a woman sixty years of age changed to black a few days before her death. The bulbs in this case were found of great size, and appeared gorged with a substance from which the hair derived its color. The white hairs that remained, on the contrary, grew from shriveled bulbs much smaller than those producing the black. This patient died of phthisis.
A very singular case, published early in the century, was that of a woman whose hair, naturally fair, assumed a tawny red color as often as she was affected with a certain fever, and returned to its natural hue as soon as the symptoms abated. Villerme alludes to the case of a young lady, sixteen years of age, who had never suffered except from trifling headaches, and who, in the winter of 1817, perceived that the hair began to fall out from several parts of her head, so that before six months were over she became entirely bald. In the beginning of January, 1819, her head became covered with a kind of black wool over those places that were first denuded, and light brown hair began to develop from the rest of the scalp. Some of this fell out again when it had grown from three to four inches; the rest changed color at different distances from its end and grew of a chestnut color from the roots. The hair, half black, half chestnut, had a very singular appearance.
Alibert and Beigel relate cases of women with blond hair which all came off after a severe fever (typhus in one case), and when it grew again it was quite black. Alibert also saw a young man who lost his brown hair after an illness, and after restoration it became red. According to Crocker, in an idiotic girl of epileptic type (in an asylum at Edinburgh), with alternating phases of stupidity and excitement, the hair in the stupid phase was blond and in the excited condition red. The change of color took place in the course of two or three days, beginning first at the free ends, and remaining of the same tint for seven or eight days. The pale hairs had more air-spaces than the darker ones. There was much structural change in the brain and spinal cord. Smyly of Dublin reported a case of suppurative disease of the temporal bone, in which the hair changed from a mouse-color to a reddish-brown; and Squire records a congenital case in a deaf mute, in whom the hair on the left side was in light patches of true auburn and dark patches of dark brown like a tortoise-shell cap; on the other side the hair was a dark brown. Crocker mentions the changes which have occurred in rare instances after death from dark brown to red.
Chemic colorations of various tints occur. Blue hair is seen in workers in cobalt mines and indigo works; green hair in copper smelters; deep red-brown hair in handlers of crude anilin; and the hair is dyed a purplish-brown whenever chrysarobin applications used on a scalp come in contact with an alkali, as when washed with soap. Among such cases in older literature Blanchard and Marcellus Donatus speak of green hair; Rosse saw two instances of the same, for one of which he could find no cause; the other patient worked in a brass foundry.
Many curious causes are given for alopecia. Gilibert and Merlet mention sexual excess; Marcellus Donatus gives fear; the Ephemerides speaks of baldness from fright; and Leo Africanus, in his description of Barbary, describes endemic baldness. Neyronis makes the following observation: A man of seventy-three, convalescent from a fever, one morning, about six months after recovery perceived that he had lost all his hair, even his eyelashes, eyebrows, nostril-hairs, etc. Although his health continued good, the hair was never renewed.
The principal anomalies of the nails observed are absence, hypertrophy, and displacement of these organs. Some persons are born with finger-nails and toe-nails either very rudimentary or entirely absent; in others they are of great length and thickness. The Chinese nobility allow their finger-nails to grow to a great length and spend much time in the care of these nails. Some savage tribes have long and thick nails resembling the claws of beasts, and use them in the same way as the lower animals. There is a description of a person with finger-nails that resembled the horns of a goat.
Neuhof, in his books on Tartary and China, says that many Chinamen have two nails on the little toe, and other instances of double nails have been reported.
The nails may be reversed or arise from anomalous positions. Bartholinus speaks of nails from the inner side of the digits; in another case, in which the fingers were wanting, he found the nails implanted on the stumps. Tulpius says he knew of a case in which nails came from the articulations of three digits; and many other curious arrangements of nails are to be found.
Rouhuot sent a description and drawing of some monstrous nails to the Academie des Sciences de Paris. The largest of these was the left great toe-nail, which, from its extremity to its root, measured 4 3/4 inches; the laminae of which it consisted were placed one over the other, like the tiles on a roof, only reversed. This nail and several of the others were of unequal thickness and were variously curved, probably on account of the pressure of the shoe or the neighboring digits. Rayer mentions two nails sent to him by Bricheteau, physician of the Hopital Necker, belonging to an old woman who had lived in the Salpetriere. They were very thick and spirally twisted, like the horns of a ram. Saviard informs us that he saw a patient at the Hotel Dieu who had a horn like that of a ram, instead of a nail, on each great toe, the extremities of which were turned to the metatarsus and overlapped the whole of the other toes of each foot. The skeleton of Simore, preserved in Paris, is remarkable for the ankylosis of all the articulations and the considerable size of all the nails. The fingers and toes, spread out and ankylosed, ended in nails of great length and nearly of equal thickness. A woman by the name of Melin, living in the last century in Paris, was surnamed "the woman with nails;" according to the description given by Saillant in 1776 she presented another and not less curious instance of the excessive growth of the nails.
Musaeus gives an account of the nails of a girl of twenty, which grew to such a size that some of those of the fingers were five inches in length. They were composed of several layers, whitish interiorly, reddish-gray on the exterior, and full of black points. These nails fell off at the end of four months and were succeeded by others. There were also horny laminae on the knees and shoulders and elbows which bore a resemblance to nails, or rather talons. They were sensitive only at the point of insertion into the skin. Various other parts of the body, particularly the backs of the hands, presented these horny productions. One of them was four inches in length. This horny growth appeared after small-pox. Ash, in the Philosophical Transactions, records a somewhat similar case in a girl of twelve.
Anomalies of the Teeth.—Pliny, Colombus, van Swieten, Haller, Marcellus Donatus, Baudelocque, Soemmering, and Gardien all cite instances in which children have come into the world with several teeth already erupted. Haller has collected 19 cases of children born with teeth. Polydorus Virgilus describes an infant who was born with six teeth. Some celebrated men are supposed to have been born with teeth; Louis XIV was accredited with having two teeth at birth. Bigot, a physician and philosopher of the sixteenth century; Boyd, the poet; Valerian, Richard III, as well as some of the ancient Greeks and Romans, were reputed to have had this anomaly. The significance of the natal eruption of teeth is not always that of vigor, as many of the subjects succumb early in life. There were two cases typical of fetal dentition shown before the Academie de Medecine de Paris. One of the subjects had two middle incisors in the lower jaw and the other had one tooth well through. Levison saw a female born with two central incisors in the lower jaw.
Thomas mentions a case of antenatal development of nine teeth. Puech, Mattei, Dumas, Belluzi, and others report the eruption of teeth in the newborn. In Dumas' case the teeth had to be extracted on account of ulceration of the tongue. Instances of triple dentition late in life are quite numerous, many occurring after a hundred years. Mentzelius speaks of a man of one hundred and ten who had nine new teeth. Lord Bacon cites the case of a Countess Desmond, who when over a century old had two new teeth; Hufeland saw an instance of dentition at one hundred and sixteen; Nitzsch speaks of one at one hundred, and the Ephemerides contain an account of a triple dentition at one hundred and twenty. There is an account of a country laborer who lost all his teeth by the time he arrived at his sixtieth year of age, but about a half year afterward a new set made their appearance. Bisset mentions an account of an old woman who acquired twelve molar teeth at the age of ninety-eight. Carre notes a case of dental eruption in an individual of eighty-five. Mazzoti speaks of a third dentition, and Ysabeau writes of dentition of a molar at the age of ninety-two. There is a record of a physician of the name of Slave who retained all his second teeth until the age of eighty, when they fell out; after five years another set appeared, which he retained until his death at one hundred. In the same report there is mentioned an old Scotchman who died at one hundred and ten, whose teeth were renewed at an advanced age after he had lost his second teeth. One of the older journals speaks of dentition at seventy, eighty-four, ninety, and one hundred and fourteen. The Philosophical Transactions of London contain accounts of dentition at seventy-five and eighty-one. Bassett tells of an old woman who had twelve molar teeth at the age of eighty-eight. In France there is recorded dentition at eighty-five and an account of an old man of seventy-three who had six new teeth. Von Helmont relates an instance of triple dentition at the same age. There is recorded in Germany an account of a woman of ninety who had dentition at forty-seven and sixty-seven, each time a new set of teeth appearing; Hunter and Petrequin have observed similar cases. Carter describes an example of third dentition. Lison makes a curious observation of a sixth dentition.
Edentulousness.—We have already noticed the association of congenital alopecia with edentulousness, but, strange to say, Magitot has remarked that "l'homme-chien," was the subject of defective dentition. Borellus found atrophy of all the dental follicles in a woman of sixty who never had possessed any teeth. Fanton-Touvet saw a boy of nine who had never had teeth, and Fox a woman who had but four in both jaws; Tomes cites several similar instances. Hutchinson speaks of a child who was perfectly edentulous as to temporary teeth, but who had the permanent teeth duly and fully erupted. Guilford describes a man of forty-eight, who was edentulous from birth, who also totally lacked the sense of smell, and was almost without the sense of taste; the surface of his body was covered with fine hairs and he had never had visible perspiration. This is probably the same case quoted in the foregoing paragraph in regard to the anomalies of hair. Otto, quoted by Sedgwick, speaks of two brothers who were both totally edentulous. It might be interesting in this connection to note that Oudet found in a fetus at term all the dental follicles in a process of suppuration, leaving no doubt that, if the fetus had been born viable, it would have been edentulous. Giraldes mentions the absence of teeth in an infant of sixteen months. Bronzet describes a child of twelve, with only half its teeth, in whom the alveolar borders receded as in age. Baumes remarks that he had seen a man who never had any teeth.
The anomalies of excessive dentition are of several varieties, those of simple supernumerary teeth, double or triple rows, and those in anomalous positions. Ibbetson saw a child with five incisors in the inferior maxillary bone, and Fanton-Touvet describes a young lady who possessed five large incisors of the first dentition in the superior maxilla. Rayer notes a case of dentition of four canines, which first made their appearance after pain for eight days in the jaws and associated with convulsions. In an Ethiopian Soemmering has seen one molar too many on each side and in each jaw. Ploucquet and Tesmer have seen five incisors and Fanchard six. Many persons have the supernumerary teeth parallel with their neighbors, anteriorly or posteriorly. Costa reports a case in which there were five canine teeth in the upper jaw, two placed laterally on either side, and one on the right side behind the other two. The patient was twenty-six years of age, well formed and in good health.
In some cases there is fusion of the teeth. Pliny, Bartholinus, and Melanthon pretend to have seen the union of all the teeth, making a continuous mass. In the "Musee de l'ecole dentaire de Paris" there are several milk-teeth, both of the superior and inferior maxilla, which are fused together. Bloch cites a case in which there were two rows of teeth in the superior maxilla. Hellwig has observed three rows of teeth, and the Ephemerides contain an account of a similar anomaly.
Extraoral Dentition.—Probably the most curious anomaly of teeth is that in which they are found in other than normal positions. Albinus speaks of teeth in the nose and orbit; Borellus, in the palate; Fabricius Hildanus, under the tongue; Schenck, from the palate; and there are many similar modern records. Heister in 1743 wrote a dissertation on extraoral teeth. The following is a recent quotation:—
"In the Norsk Magazin fur Laegevidenskaben, January, 1895, it is reported that Dr. Dave, at a meeting of the Medical Society in Christiania, showed a tooth removed from the nose of a woman aged fifty-three. The patient had consulted him for ear-trouble, and the tooth was found accidentally during the routine examination. It was easily removed, having been situated in a small depression at the junction of the floor and external wall of the nasal cavity, 22 mm. from the external nares. This patient had all her teeth; they were placed somewhat far from each other. The tooth resembled a milk canine; the end of the imperfect root was covered with a fold of mucous membrane, with stratified epithelium. The speaker suggested that part of the mucous membrane of the mouth with its tooth-germ had become impacted between the superior and premaxillary bones and thus cut off from the cavity of the mouth. Another speaker criticised this fetal dislocation and believed it to be due to an inversion—a development in the wrong direction—by which the tooth had grown upward into the nose. The same speaker also pointed out that the stratified epithelium of the mucous membrane did not prove a connection with the cavity of the mouth, as it is known that cylindric epithelium-cells after irritative processes are replaced by flat ones."
Delpech saw a young man in 1829 who had an opening in the palatine vault occasioned by the extraction of a tooth. This opening communicated with the nasal fossa by a fracture of the palatine and maxillary bones; the employment of an obturator was necessary. It is not rare to see teeth, generally canine, make their eruption from the vault of the palate; and these teeth are not generally supernumerary, but examples of vice and deviation of position. Fanton-Touvet, however, gives an example of a supernumerary tooth implanted in the palatine arch. Branch a describes a little negro boy who had two large teeth in the nose; his dentition was otherwise normal, but a portion of the nose was destroyed by ulceration. Roy describes a Hindoo lad of fourteen who had a tooth in the nose, supposed to have been a tumor. It was of the canine type, and was covered with enamel to the junction with the root, which was deeply imbedded in the side and upper part of the antrum. The boy had a perfect set of permanent teeth and no deformity, swelling, or cystic formation of the jaw. This was clearly a case of extrafollicular development and eruption of the tooth in an anomalous position, the peculiarity being that while in other similar cases the crown of the tooth shows itself at the floor of the nasal cavity from below upward, in this instance the dental follicle was transposed, the eruption being from above downward. Hall cites an instance in which the right upper canine of a girl erupted in the nose. The subject showed marked evidence of hereditary syphilis. Carver describes a child who had a tooth growing from the lower right eyelid. The number of deciduous teeth was perfect; although this tooth was canine it had a somewhat bulbulous fang.
Of anomalies of the head the first to be considered will be the anencephalous monsters who, strange to say, have been known to survive birth. Clericus cites an example of life for five days in a child without a cerebrum. Heysham records the birth of a child without a cerebrum and remarks that it was kept alive for six days. There was a child born alive in Italy in 1831 without a brain or a cerebellum—in fact, no cranial cavity—and yet it lived eleven hours. A somewhat similar case is recorded in the last century. In the Philosophical Transactions there is mentioned a child virtually born without a head who lived four days; and Le Duc records a case of a child born without brain, cerebellum, or medulla oblongata, and who lived half an hour. Brunet describes an anencephalous boy born at term who survived his birth. Saviard delivered an anencephalous child at term which died in thirty-six hours. Lawrence mentions a child with brain and cranium deficient that lived five days. Putnam speaks of a female nosencephalous monster that lived twenty-nine hours. Angell and Elsner in March, 1895, reported a case of anencephaly, or rather pseudencephaly, associated with double divergent strabismus and limbs in a state of constant spastic contraction. The infant lived eight days. Geoffroy-Saint-Hilaire cites an example of anencephaly which lived a quarter of an hour. Fauvel mentioned one that lived two hours, and Sue describes a similar instance in which life persisted for seven hours and distinct motions were noticed. Malacarne saw life in one for twelve hours, and Mery has given a description of a child born without brain that lived almost a full day and took nourishment. In the Hotel-Dieu in Paris in 1812 Serres saw a monster of this type which lived three days, and was fed on milk and sugared water, as no nurse could be found who was willing to suckle it.
Fraser mentions a brother and sister, aged twenty and thirty, respectively, who from birth had exhibited signs of defective development of the cerebellum. They lacked power of coordination and walked with a drunken, staggering gait; they could not touch the nose with the finger when their eyes were shut, etc. The parents of these unfortunate persons were perfectly healthy, as were the rest of their family. Cruveilhier cites a case of a girl of eleven who had absolutely no cerebellum, with the same symptoms which are characteristic in such cases. There is also recorded the history of a man who was deficient in the corpus callosum; at the age of sixty-two, though of feeble intelligence, he presented no signs of nervous disorder. Claude Bernard made an autopsy on a woman who had no trace of olfactory lobes, and after a minute inquiry into her life he found that her sense of smell had been good despite her deficiency.
Buhring relates the history of a case somewhat analogous to viability of anencephalous monsters. It was a bicephalous child that lived thirty-two hours after he had ligated one of its heads.
{footnote} The argument that the brain is not the sole organ of the mind is in a measure substantiated by a wonderful case of a decapitated rooster, reported from Michigan. A stroke of the knife bad severed the larynx and removed the whole mass of the cerebrum, leaving the inner aspect and base of the skull exposed. The cerebrum was partly removed; the external auditory meatus was preserved. Immediately after the decapitation the rooster was left to its supposed death struggles, but it ran headless to the barn, where it was secured and subsequently fed by pushing corn down its esophagus, and allowing water to trickle into this tube from the spout of an oil-can. The phenomena exhibited by the rooster were quite interesting. It made all the motions of pecking, strutted about, flapped its wings, attempted to crow, but, of course, without making any sound. It exhibited no signs of incoordination, but did not seem to hear. A ludicrous exhibition was the absurd, sidelong pas seul made toward the hens.
Ward mentions an instance of congenital absence of the corpora callosum. Paget and Henry mention cases in which the corpora callosum, the fornix, and septum lucidum were imperfectly formed. Maunoir reports congenital malformation of the brain, consisting of almost complete absence of the occipital lobe. The patient died at the twenty-eighth month. Combettes reports the case of a girl who died at the age of eleven who had complete absence of the cerebellum in addition to other minor structural defects; this was probably the case mentioned by Cruveilhier.
Diminution in volume of the head is called microcephaly. Probably the most remarkable case on record is that mentioned by Lombroso. The individual was called "l'homme-oiseau," or the human bird, and his cranial capacity was only 390 c.c. Lombroso speaks of another individual called "l'homme-lapin," or man-rabbit, whose cranium was only slightly larger than that of the other, measuring 490 mm. in circumference. Castelli alludes to endemic microcephaly among some of the peoples of Asia. We also find it in the Caribbean Islands, and from the skulls and portraits of the ancient Aztecs we are led to believe that they were also microcephalic.
Two creatures of celebrity were Maximo and Bartola, who for twenty-five years have been shown in America and in Europe under the name of the "Aztecs" or the "Aztec children". They were male and female and very short, with heads resembling closely the bas-reliefs on the ancient Aztec temples of Mexico. Their facial angle was about 45 degrees, and they had jutting lips and little or no chin. They wore their hair in an enormous bunch to magnify the deformity. These curiosities were born in Central America and were possibly half Indian and Negro. They were little better than idiots in point of intelligence.
Figure 92 represents a microcephalic youth known as the "Mexican wild boy," who was shown with the Wallace circus.
Virchow exhibited a girl of fourteen whose face was no larger than that of a new-born child, and whose head was scarcely as large as a man's fist. Magitot reported a case of a microcephalic woman of thirty who weighed 70 pounds.
Hippocrates and Strabonius both speak of head-binding as a custom inducing artificial microcephaly, and some tribes of North American Indians still retain this custom.
As a rule, microcephaly is attended with associate idiocy and arrested development of the rest of the body. Ossification of the fontanelles in a mature infant would necessarily prevent full development of the brain. Osiander and others have noticed this anomaly. There are cases on record in which the fontanelles have remained open until adulthood.
Augmentation of the volume of the head is called macrocephaly, and there are a number of curious examples related. Benvenuti describes an individual, otherwise well formed, whose head began to enlarge at seven. At twenty-seven it measured over 37 inches in circumference and the man's face was 15 inches in height; no other portion of his body increased abnormally; his voice was normal and he was very intelligent. He died of apoplexy at the age of thirty.
Fournier speaks of a cranium in the cabinet of the Natural History Museum of Marseilles of a man by the name of Borghini, who died in 1616. At the time he was described he was fifty years old, four feet in height; his head measured three feet in circumference and one foot in height. There was a proverb in Marseilles, "Apas mai de sen que Borghini," meaning in the local dialect, "Thou hast no more wit than Borghini." This man, whose fame became known all over France, was not able, as he grew older, to maintain the weight of his head, but carried a cushion on each shoulder to prop it up. Fournier also quotes the history of a man who died in the same city in 1807 at the age of sixty-seven. His head was enormous, and he never lay on a bed for thirty years, passing his nights in a chair, generally reading or writing. He only ate once in twenty-four or thirty hours, never warmed himself, and never used warm water. His knowledge was said to have been great and encyclopedic, and he pretended never to have heard the proverb of Borghini. There is related the account of a Moor, who was seen in Tunis early in this century, thirty-one years of age, of middle height, with a head so prodigious in dimensions that crowds flocked after him in the streets. His nose was quite long, and his mouth so large that he could eat a melon as others would an apple. He was an imbecile. William Thomas Andrews was a dwarf seventeen years old, whose head measured in circumference 35 inches; from one external auditory meatus to another, 27 1/4 inches; from the chin over the cranial summit to the suboccipital protuberance, 37 1/2 inches; the distance from the chin to the pubes was 20 inches; and from the pubes to the soles of the feet, 16; he was a monorchid. James Cardinal, who died in Guy's Hospital in 1825, and who was so celebrated for the size of his head, only measured 32 1/2 inches in head-circumference.
The largest healthy brains on record, that is, of men of prominence, are those of Cuvier, weighing 64 1/3 ounces; of Daniel Webster, weighing 63 3/4 ounces (the circumference of whose head was 23 3/4 inches); of Abercrombie, weighing 63 ounces, and of Spurzheim, weighing 55 1/16 ounces. Byron and Cromwell had abnormally heavy brains, showing marked evidence of disease.
A curious instance in this connection is that quoted by Pigne, who gives an account of a double brain found in an infant. Keen reports finding a fornix which, instead of being solid from side to side, consisted of two lateral halves with a triangular space between them.
When the augmentation of the volume of the cranium is caused by an abundant quantity of serous fluid the anomaly is known as hydrocephaly. In this condition there is usually no change in the size of the brain-structure itself, but often the cranial bones are rent far asunder. Minot speaks of a hydrocephalic infant whose head measured 27 1/2 inches in circumference; Bright describes one whose head measured 32 inches; and Klein, one 43 inches. Figure 93 represents a child of six whose head circumference was 36 inches. Figure 94 shows a hydrocephalic adult who was exhibited through this country. |
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