CASE OF A BEARDED WOMAN LOUIS A. DUHRING, M. D., Professor of Skin Diseases in the Hospital of the University of Pennsylvania. (re-printed from the ARCHIVES OF DERMATOLOGY, APRIL, 1877.) NEWYORK: G. P. PUTNAM’S SONS. 182 FIFTH AVENUE. 1877. CASE OF A BEARDED WOMAN LOUIS A. DUHRING, M. D., Professor of Skin Diseases in the Hospital of the University of Pennsylvania. (re-printkd from the archives of DERMATOLOGY, APRIL, 1577.) NEWYORK: G. p. putnam;’s sons., 182 FIFTH AVENUE. 1877. dr. du wring’s case of bearded, woman . CASE OF A BEARDED WOMAN. THE case whose portrait accompanies these notes was recently shown to the medical class of the University of Pennsylvania, and, in connection with remarks upon hypertrophy of the hair in general, was made the subject of a clinical lecture. The growth is such an unusual one, and the deformity so striking, that it appears to me to be worthy of detailed description and a place upon rec- ord. Previous History.—Her name is Mrs. Viola M . She was born in the town of Wilcox, Elk Co., Pennsylvania, in 1854, and is now, therefore, twenty-three years old. At birth, according to the state- ment of her parents, both of whom are living, she had a very light, whitish downy growth of hair over the regions of the face that are now hirsute. The rest of the face and the body showed no sign of hair. During the first month this in a measure wore off, but a few months later returned and began to grow stronger. The scalp pos- sessed no more hair than is common to the majority of children at birth. At the age of three years, we are informed, considerable hair was present over the sides of the cheeks, after the manner of whiskers, as light, flaxen lanugo. The hair of the scalp at this age was light brownish in color. Throughout her childhood she was weakly, and her general condition below the average. She was frail, spare and delicate, and ailed from time to time with various complaints. The hair upon her face, however, continued to in- crease in quantity and in length perceptibly from year to year. As a child she was extremely modest, shy, and diffident, and never ex- hibited any signs of precocity. At about the age of ten the hair of the face began to grow more vigorously, the cheeks, chin and upper portion of neck showing an abundant production. The hair upon the upper lip now first man- ifested itself in a notable manner, and in a short time became 4 brownish in color. At twelve, the hair of the scalp was quite long, extending to the hips, but it was by no means thick, and was not considered a full head of hair. She was at this period seized with a severe fever, during the course of which the hair of the scalp was sacrificed and cut short. After recovery it began to grow again, but only very slowly, and has never reached its former length. Menstruation set in naturally at the age of fourteen, and was un- attended by any unusual phenomena. From this date her general health improved, and in the course of a few years from a delicate child she appeared quite hearty. The establishment of this func- tion, which at once became regular and normal in all respects, did not exert any perceptible influence upon the growth of the hair of the face. It neither grew more rapidly nor did it become coarser in texture. Upon this point I have questioned her closely, but she is quite sure of the correctness of the statement. With puberty hair first manifested itself in the axillae and on the pubes. It also now appeared upon certain regions of the trunk, and upon the ex- tremities, in the form of stout lanugo. The mammae enlarged and soon attained the development common to the majority of young women. They were neither full, nor on the other hand small, but, she thinks, of medium size. At sixteen, two years after the establish- ment of menstruation, another start in the growth of the hair of the face took place, the hair becoming more abundant, longer, stouter, and darker in color. At eighteen it had reached its greatest devel- opment,—the condition in which it now exists. It has undergone no appreciable change during the last five years. It has never been cut, shaved, nor in any way operated upon, as, for example, with depilatories. She was married at the age of seventeen-and-a-half to her pres- ent husband (who, it may be remarked, possesses no peculiarities),, by whom she has had two children, one a boy, the other a girl. They were born at full term, the labor with both having been easy and perfectly normal. The children were well developed and showed no signs whatsoever of the mother’s deformity. They lived to the ages of two and four years, respectively, and died within a short time of each other, in September last, of scarlatina. Up to the period of their decease they had manifested no tendency to any unusual development of hair, either upon the scalp or elsewhere. During gestation no changes in the growth or condition of the hair were observed. She was able to nurse her children for about a month, when the milk began to fail. It so happened with both children. Her voice has always been feminine. It has not been in any way remarkable. The throat for some years has been irritable and liable to colds accompanied with tenderness and a slight cough. Four months ago she contracted a most severe cold which settled about her throat, and has persistently remained, attended with hoarseness and pain in speaking. Present Condition.—She is a woman of small stature, meas- uring in height not more than five feet one or two inches, and of slen- 5 der frame, but well formed and proportioned. Her head is of medium size, rounded and shapely. The face is a refined one, ovalish in form, with a moderately high forehead, straight, delicately chiselled nose, small, pleasing mouth, with thin lips, and a retiring chin. The hair is black ; the eyes dark brown. The complexion is a clear brunette, the cheeks showing considerable color. The skin of the face is remarkably soft and smooth, and singularly free of all gland- ular disorder. The ears are well shaped and exhibit no peculiarities, either as to form, in the way of points, or in the growth of hair. The larynx is not larger than usual upon women of her build and age, and is not prominent. Owing to the inflammatory and painful laryngeal affection, the voice at present is feeble and hoarse. Dr. J. Solis Cohen and myself have made an examination of the larynx, and find the vocal cords to be intensely red and hyperaemic. The teeth are in a remarkably fine state of preservation, and are perfect as to form, number, and order. Her natural constitution, or physique, is by no means strong. The chest is narrow and hollow. The mammae are of equal size, small and flat, and look as though they might belong to a frail, delicate young woman who had but recently entered upon puberty. The nipples, and areolae with their papillae, are unusually small, are pinkish in color, and but slightly pigmented, and have a somewhat masculine appearance as to form, size, and color. She assures me that these structures have changed greatly of late, and that whereas they now exist as I have represented them they were formerly womanly, and that the mammary glands were of average size. As she remarks, “the chest seems to have contracted and the breasts to have shrunken.” The arms and legs are feminine in shape, and are slender and spare. The hands and feet, fingers and toes, are small and womanly, and possess no more hair than is often met with in women. The nails are perfectly formed and present nothing un- usual. The integument is everywhere, with the exception of the face, dry, thin, and wanting in adipose tissue. She informs me that a marked unaccountable change in her general physique has taken place during the last year, and that she has lost much flesh, as much, latterly, as ten or fifteen pounds. She is becoming sparer month by month, and this without any apparent cause, for her health is fair, her appetite good, and the various functions quite regular. Menstruation still continues to take place normally in all respects. Her character is strictly womanly, all her tastes being remarka- bly feminine and domestic. She possesses a quiet, composed man- ner, and is reserved, taciturn, and modest in disposition. Her fa- cial expression, without being melancholic, is habitually thoughtful. Growth of Hair.—Upon the scalp the hair is short, when permitted to hang loose, reaching only to the shoulders, and is quite thin. It is black, has a brilliant lustre, is naturally oily, and is of fine texture. It is equally distributed over the scalp, and does not grow in the form of tufts, or in any way irregularly. Around the margin, it terminates on all sides normally. It neither encroaches 6 in front upon the forehead, nor behind upon the neck, nor is it found upon the auricle or within the external auditory canal. It even ceases abruptly about the temporal region, and does not run into the side whisker. The hair of the scalp is distinct and of another kind from that of the whisker. The eyebrows and eyelashes are full, but by no means heavy. The upper lip, cheeks, chin and sub-maxillary region are the seat of hair, in the form of full mustache and beard, which involves ex- actly the same regions as in the male. The hairs of the mustache are about a half inch long, of uniform length, pointed, and fine in texture. Those of the whisker and beard are four or five inches in length, curly, abundant and thickly set, and likewise of fine quality, although they would scarcely be termed soft and silky. Both are black in color, the central portion of the beard showing a dark reddish-brown shade. Viewed in its entirety the beard would be termed full, thick and handsome, and is such as is not unfrequently met with upon men who have never shaved. It grows evenly and uniformly thick on all sides, although fullest at the angles of the lower jaw. The hairs come out normally from the follicles, and tend to curl slightly. Upon the cheeks, over the malar bones, the hair, as is ordinarily the case in men, terminates in lanugo, but does not encroach beyond the region of the malar bones. The nose and forehead are singularly free of hair. Extending from shoulder to shoulder, over the back, there exists sufficient hair to constitute a diffused hairy patch, about the width of a hand. It is composed of thin, soft, blackish hair, about an inch in length. The hairs are not very closely set, and are not so numerous but that the skin may be readily seen through. They are thickest over the region of the upper dorsal vertebrae. The growth ceases, with straggling hairs, on either side, upon the shoulders. What is quite curious, there does not exist any con- nection between this patch on the back and the hair of the scalp ; fqr over the lower cervical vertebrae there is a space several inches in width which is entirely devoid of hair other than fine lanugo. The whole of the back, however, on either side, from the scalpulae downwards, is covered sparsely with the same kind of hair as about the shoulders, but not so stout nor so thickly set. The hairs start from either side of the spinal column, and take a course downwards and forwards around the sides of the thorax, covering the lateral portions of the trunk. The vertebral region itself is almost entirely free of hair. The hairs lie close to the skin, and are uniformly dis- tributed over the surface. The axillae possess no more hair than is ordinarily met with in woman. Over the upper portion of the chest, below the clavicles, are noticed a few straggling, fine hairs, but they are not sufficie v numerous to attract attention. The mammae are smooth and devoid of hair ; nor are there any short, stout hairs about the areolae of the nipples, where they might be expected to exist. Upon the abdo- men there is a small, finger-nail sized, pigmentary mole, but it is said not to be hairy. The umbilicus is free from hair. The pubes, lam 7 assured, possess no more hair than is commonly there encountered in women ordinarily hirsute ; nor, from the condition of the axillae, should we expect to find an undue amount of hair on the pubes. The thighs and legs are slightly hairy, the hairs being long and thin, but not as numerous nor as strong as usually met with upon men. The arms, however, from the shoulders to the wrists are somewhat more hairy, but not to the extent that they would be con- sidered in any way remarkable. The hands and feet have the usual amount of lanugo, a shade stronger and darker than com- monly seen in women. Upon the arms, thighs and legs there ex- ists a form of lichen pilaris,—a condition characterized by dryness and roughness of the skin and an obstruction of the openings of the hair follicles, with little conical masses of dried epithelium. The color of the integument of the body and extremities is yellowish- brown or swarthy. Family History.—The parents of our subject are both living and are well known in the community where they reside. They are respectable country people, and are hearty and in every way normally developed individuals. Their parents (the grandparents of our case) were likewise healthy and normally constituted people. Neither generation manifested any unusual growth of hair ; nor is any one in the family or among the relatives known to have shown any tendency to the development of hairy growths. Our patient has two brothers, one aged twenty, the other eleven, and one sister, nine years old, comprising the family of her parents, none of whom present any peculiarities. They exhibit no unusual growth of hair either upon the head or body. The eldest brother, until he arrived at the age of fifteen, is said to have had a remarkably smooth face. The sister likewise offers no unusual amount of hair. Having described the most noteworthy features in connection with the history and present condition of the case, I desire to call attention briefly to several of the more prominent points which combine to make it one of more than usual interest. Although the development of hair upon the trunk and extremi- ties is in itself anomalous, and entitled to special remark, yet, viewed as a deformity, this must be regarded as of insignificance in comparison with that upon the face. Upon the general surface, with the exception of the back, it is simply an augmented growth of lanugo, such as is not very rarely seen upon middle-aged women, but upon the face it is altogether an abnormal and moreover an ex- cessive production. At the same time it must be conceded, I think, that there exists a close connection between the hirsuties of the trunk and extremities and that of the face, a point to which I shall presently revert. On the other hand, there does not appear to be any connecting link between the development of the hair in regions where it is normal in woman, as on the scalp, in axillae and on pubes, and that of the face and trunk. It is important to note, in the first place, that the hair of the face was a congenital condition, a certain amount of lanugo, it will be remembered, having been present at birth. Upon other regions 8 of the body, excepting the scalp, there was no trace of hair ; nor, ac- cording to the statement made, did any make its appearance until puberty, when it began to show itself upon the trunk and limbs and other regions usually hairy, namely, axillae and pubes. The fact of the hirsuties of the face being congenital, explains, in a measure, several of the peculiarities of the case. Had, for example, the hair of the face been an acquired growth, appearing for the first time at puberty or later, and dependent upon sexual development, there would without doubt have been other decided masculine char- acters present. As has been observed, however, the hair follicles and hairs of the face were at birth already well-formed and in an advanced state of development, and began to grow at once and continued to grow for some years at an uniform pace, subject ap- parently to the same influences as those which regulated the growth of the hair of the scalp. It has been noticed that the general health was much below the average throughout childhood, and that physical bodily develop- ment took place very tardily. I cannot think, however, that this was in any way dependent upon the abnormal growth of hair, but that it was rather an accidental circumstance, resulting from other and quite different causes. This observation seems to be fully sub- stantiated by the fact that the period of puberty exerted no particu- lar effect upon the growth of the hair of the face, and this point seems to me to be one of the most noteworthy in the case. As has been stated, the hair of the face increased quite unifd’rmly until about the age of ten, when it grew rapidly for a short time. At fourteen puberty occurred, but without any appreciable increase of the hair upon the face, and at sixteen there was again a rapid growth, which continued for about a year, when it attained its great- est profusion. It is also to be noted that puberty did not occur be- fore the usual age, an event which might have been looked for considerably earlier than the usual period ; nor was it accompanied by any pain, difficulty, or general disturbance of the system. Con- trary to what we should have expected, moreover, the flow became at once normal. All of these points go to show conclusively, 1 think, the complete independence of the hairy growth of the face of sexual development. The perfectly natural increase of the the scalp, axillae and pubes is also an occurrence worthy of remark, for we should have nat- urally anticipated a more or less excessive growth certainly upon the head. These regions were manifestly not under the same influence as that which regulated the growth of hair elsewhere. They seem to have entirely escaped this force, for the hair upon these parts has at no time offered any peculiarities. Contrary to what is commonly observed in similar cases, the hair did not manifest any disposition to encroach upon the forehead or upon the ears, nor to extend itself down the nape of the neck and over the vertebral column. All of these localities remained free. Concerning the general hirsuties of the body, if we accept the statement of the patient as to its growth as being correct (a point, 9 however, that I have from the first been inclined to doubt), we must concede that it was occasioned by the same normal influences which calls forth the hair on usually hairy parts, as, for example, in the axillae and on the pubes. But, as I have before intimated, it seems to me much more likely that it manifested itself early in life rather than at puberty, perhaps so slightly as to have been over- looked, and that therefore it was, we may almost say, congenital; just as in the case of ichthyosis, where, as we know, the deformity very often does not show itself until some months after birth. The non-existence of circumscribed patches of hair or tufts, and of hairy moles, one or more of which we should rather anticipate meeting in such a case, may also be referred to as being unusual. The most interesting feature, however, is the complete absence of all signs of masculinity, traces of which we should naturally look for and expect to find either in the physical structure or in the dis- position. But, as I have stated quite fully in the notes, the contour of the body, the form of the pelvis, limbs, hands and feet, the features of the face, the larynx and the voice are all strictly femi- nine. Although, as described, the mammas are small and flat, ac- cording to her own statement as well as that of her husband, this condition is of recent date and has taken place gradually within the last year or two. As we have recorded in the history, the breasts at puberty though not large were by no means small, and at the birth of her children contained sufficient milk to nourish them for a month or longer. Whether this marked diminution in their size is to be attributed to the failure of her general health, which has been accompanied by decided loss of weight, or whether an important physical change, attended by the establishment of certain mascu- line traits, is about taking place, cannot as yet be determined. At present, however, the atrophy of the breasts is the only sign of the kind to be noted. I may also revert, in passing, to the unusual regularity of the teeth, for, as is well known, observers have from time to time stated, whether correctly or not, that it is common to observe in congenital hirsuties more or less irregularity and deficiency of these structures. There is certainly no reason to suppose that any hermaphroditic element exists in the constitution of our case. Beyond the hirsu- ties of the face, I have been able to find no virile characters. The circumstance of menstruation having occurred with regularity and normally since the age of fourteen, and that she has conceived and borne two children at brief intervals, clearly determines that the female sexual characters are fully and perfectly developed. The entire absence of any hereditary history is also.a matter of interest, for, although the non-existence of such influence is not un- frequently observed in similar cases, it would certainly have been more in accord with the laws of heredity had there been some trace of hirsuties in parents or in grandparents. But what is still more remarkable is that two children should have been borne to the mother without having manifested, up to the age at least of four years, any similar peculiarity. Upon the subject of there having been any “maternal impres- sion ” during pregnancy in our case, we learn no facts which can be regarded as being of any value. During the mother’s pregnancy there lived close by a woman who had a small hairy mole upon the cheek, and who was the subject of remark on the part of the mother, but I am unable to learn that she placed any stress upon this circumstance, or that she even attributed the deformity of her daughter to her having daily seen this woman. The mother, indeed, offers no suggestion by which she can, even to her own mind, ac- count for the production. In regard to the question which was asked me by the patient, whether the growth of the face could be successfully and perma- nently removed (and I need not add that it is to her the source of intense mortification and distress), I would say that the only justifi- able means at our command is palliative, consisting in either the daily use of the razor or in the employment of a depilatory powder. The recommendation of one or the other must depend upon the circumstances peculiar to the case. In the case before us, consider- ing the extensive surface to be operated upon, I should favor the employment of the razor. The portrait which accompanies these remarks has been drawn on stone from a photograph taken quite recently, and is an accurate representation of the beard as it now exists.