THE PIGMENTARY SYPHILIDE BY f R. W. TAYLOR, M. D. CLINICAL PROFESSOR OF VENEREAL DISEASES AT THE COLLEGE OF PHYSICIANS AND SURGEONS, NEW YORK REPRINTED FROM THE NEW YORK MEDICAL JOURNAL FOR FEBRUARY 18, 1898 NEW YORK D. APPLETON AND COMPANY 1893 Fig. I DrTayloris Article on the Pigmentary Syphilide. THE PIGMENTARY SYPHILIDE BY E. W. TAYLOE, M. D. CLINICAL PROFESSOR OF VENEREAL DISEASES AT THE COLLEGE OF PHYSICIANS AND SURGEONS, NEW YORK REPRINTED FROM THE NEW YORK MEDICAL JOURNAL FOR FEBRUARY 18, 1893 NEW YORK D.APPLETON AND COMPANY 1893 Copyright, 1893, BY D. APPLETON AND COMPANY. THE PIGMENTARY SYPHILIDE* The history of the pigmentary syphilide is a most pe- culiar one. It is an affection which at first was clearly and sharply described, but which in the course of time has been rendered so obscure that to-day very few have clear and precise ideas as to its course and its nature. In the whole range of syphilography there is not a like instance in which the knowledge of a manifestation of syphilis has become so progressively obscure and in which so much confusion has been interjected by reason of the successive additions to its literature by many writers. In the sixties we knew what the pigmentary syphilide was as a result of the writings of Hardy, Fournier, Pillon, and Tanturri. To- day this well-marked and peculiarly characteristic affection is so little understood that it is confounded with the pig- mentations and the leucodermatous conditions left as a result of previous syphilitic processes. The writings of Neisser, Riehl, Ehrmann, Poelchen, and Szadeck have had much to do with the obscuration of this question, for these authors regard any pigmentation or leucodermatous condition pri- mary or secondary to a previous syphilitic process as ex- amples of the pigmentary syphilide. We can not too strongly insist upon the necessity of holding fast to the postulate that the pigmentary syphilide is a unique, well-marked affection, having a sharply defined pathological basis and a course attended by well demon- strated morphological changes. As a corollary of this, I may add that secondary pigmentations and leucodermatous conditions occurring in the course of syphilis, as relics or sequelae of lesions chiefly secondary, are in no sense exam- ples of the pigmentary syphilide ; they are simply dischro- matous accidents and not sharply defined essential affec- tions. The reasons why this confusion has been induced are many, and the chief ones are the following: 1. Many of the writers have had little experience in the study of syphilis, and have written in a dogmatic manner from the observation (and that usually very limited as to time) of one or perhaps two cases. 2. Conclusions have been drawn from clinical appear- ances presented at various stages in the progress of the af- fection, which, being of long duration and presenting at dif- ferent periods varying pictures, can not be well understood by any one unless he has had his case or cases under his observation during the whole period of development, evo- lution, and involution of the affection. 3. With one exception (Maieff), authors have studied the question from a histo-pathological basis in a hap- hazard way, but have been none the less dogmatic in their conclusions. Thus no observer until Maieffs time studied the disease microscopically step by step in accordance with its natural evolution. On the contrary, sections of skin were made indiscriminately in cases of secondary pigmenta- tions and leucodermatous conditions, and perhaps in cases of the true pigmentary syphilide. In no instance is any distinction observed. In this way discrepancies have been produced, and flat contradictions and anomalies have re- sulted. 4. Every pigmentation in a syphilitic, recent or old, is called the pigmentary syphilide, and the latter is thus de- prived of its essential character. For many years I have carefully studied the affection upon a large number of patients, in many instances during its whole course, and I venture to present my conclusions, now feeling confident that they are correct and with the hope that the light on this subject here offered may extend and do something to dissipate the prevailing doubt, ob- scurity, and confusion. The primordial pigmentary anomalies due to syphilis consist essentially in a superpigmentation, which may in whole or in part be replaced by a corresponding loss of color or leucodermatous condition. This primordial hyper- pigmentation is the essential pigmentary syphilide ; all other discolorations are secondary processes and in no manner entitled to be classed as pigmentary syphilide. The pigmentary syphilide is seen in three well-marked and quite distinct conditions : 1. In the form of spots or patches of various sizes. 2. As a diffuse pigmentation of greater or less intensity, which sooner or later becomes the seat of leucodermatous changes in the shape of small spots which gradually in- crease in size. This is the retiform pigmentary syphilide— the syphilide pigmentaire a dentelles of Fournier. 3. In an abnormal distribution of the pigment of the * Read before the American Association of Genito-urinary Surgeons, June 21, 1892. THE PIGMENTARY SYPHILIDE. 4 skin, in which, owing to the lack of or crowding out of the pigment in places, they become whiter, while the parts in- volved in the abnormal distribution become darker ; in this way a dappled appearance is presented. In this form there is probably no excess of pigment; it is seemingly unequally distributed throughout the tissue expanse. This form has been termed the marmoraceous, from its resemblance to some forms of marble in which there is an intimate inter- blending of light and darker colors. This marmoraceous pigmentary syphilide is not common, and it is peculiarly liable, by reason of its delicacy of tone and tint, to pass unobserved. The pigmentary syphilide in the form of spots or patches consists of round, oval, or irregular plaques, which may have sharply defined borders or their margins may be den- tated or jagged. Their color varies from a light-brown cafe au lait to even a quiet deep brown tint. They are unaffected by pressure and the condition of the circulation. In persons of light and delicate skin they may be very faint in tint and perhaps only perceptible in oblique light. In Fig. 3 an ad- mirable picture of the spot-form pigmentary syphilide is shown. In this case the pigmentation was very deep. It was under my observation for a long time, during which I observed its evolution as pin-head-size spots, which in- creased in area until they reached the size shown in the figure. In this woman this eruption appeared toward the end of the first year of syphilis. Prior to its onset the neck had not been invaded by syphilitic lesions of any form ; consequently this eruption was not a posthumous ex- pression of an antecedent eruption. In this form of pigmentary syphilide it is common to see the uneven distribution of the pigmentation ; sometimes the color is deeper at the margin. Commonly there is no involvement of the intervening skin, though sometimes the hyperchromatous condition produces the illusion that the unaffected skin is whiter than normal. These pigmented spots may remain unchanged and indolent for months, particularly in cold weather. In the course of time they show evidence of fading and they slowly disappear. The process of involution may begin at the margin and extend centripetally, or it may take place in the whole morbid area. In some cases colorless patches are left after the disappearance of the pigmentation ; there is then produced a secondary or pseudo-leucoderma. Now, if a case is seen only in this stage, I can well understand an observer reach- ing the conclusion that the process was an atrophic one; consequently it is easy to see why so much is written upon syphilitic leucoderma and syphilitic vitiligo. These ex- pressions clearly show the want of a full knowledge of the disease, and that the observer has only acquainted himself with its stage of decline. In most cases the skin retains its normal appearance after the full involution of this syph- ilide. In Figs. 4 and 5 is well shown a disseminated erup- tion of pigmented spots which followed a papular syphilide. This is an excellent illustration of secondary pigmentation. The second form of pigmentary syphilide—the lace or retiform variety—is far more common than the previous form. More or less slowly and even rapidly the sides of the neck become discolored, the tint being that of cafe au lait, or even of decided yellowish brown. The most common site of this eruption is on the sides of the neck and perhaps on the back of the neck. The patients usually say that they noticed, or were told, that their necks were getting or had got dirty. Intelligent and observant patients will very often distinctly state that their trouble began with a browning of the skin, and they will state positively that there was no intermingling of white spots. From the neck this eruption may extend more or less extensively over the trunk, mostly anteriorly or down the arms. I have never seen it go up on the face. In many cases this eruption passes unnoticed and may be attributed to the action of the sun, to irritation, or even to uncleanliness. When the pigmented patch has involved more or less of the sides of the neck a peculiar change will be observed in it—namely, the development of whitish spots which may be taken for leucoderma. Scattered irregularly over the pigmented sur- face close observation will show a few or many minute white specks, which in a short time, particularly in hot weather, will be large enough to present definite shapes, which may be round, oval, linear, or irregular. These white spots gradually grow, and in many instances the neck is largely covered with them before the patient knows of any change having taken place. They then say or are told that their necks are growing white. Undoubtedly many a doctor, upon this information being given him, has con- cluded that he has a case of leucoderma before him. Sometimes the white patches are distinctly lighter than the normal skin; in other instances the contrast between dark and light is illusory, and there is really no difference in color between the so-called leueodermatous patches and the unaf- fected skin. The white spots may or may not be sharply marginated, in some cases the line of margination being clear and sharp and in others indistinct. I have never seen the thin, filmy, superpigmented area around white patches of true pigmentary syphilide which we see so clearly and so commonly at the circumference of patches of leucoderma or vitiligo, as it is called. This point, in my judgment, is of diagnostic import, and is explained by the pathology of the disease to be considered further on. The tendency of the white spots to extend necessarily means the diminution of the brown background. In this way we have various pictures presented, as shown in Figs. 1 and 2. In this way is produced a dappled appearance, which war- rants the name for this eruption at this time of the dappled syphilide. Toward the final stage of the disease the pre- ponderance of the white spots leaves only round, oval, or wavy lines or strands of brown pigment, which gives the appearance of lace with large meshes, the interstices being formed by the white spots, which are round, oval, gyrate, linear, or irregular. In this way the skin in the course of months, and in some cases of a year or more, gradually seemingly returns to its normal condition. In the study of these cases I have sometimes seen during the activity of the process a mild and ephemeral hyperaemia, which might oasily have escaped observation, and the question suggests itself to my mind whether or not a mild form of conges- tion may precede the hyperpigmentation. In Fig. 1 this form of the pigmentary syphilide is well THE NEW YORK MEDICAL JOURNAL, FEBRUARY 18, 1893. Fig. 2.—Spotted form of pigmentary syphilkle. Fig. 3.— Retiform pigmentary syphilide. Fig. 4. Fig. 5. Pigmentations secondary to the papular syphilide. THE PIGMENTARY SYPHILIDE. 5 shown covering the neck, the anterior and lateral parts of the trunk, and the anterior surface of the arms as far as the insertion of the deltoids. This case is remarkable and ex- ceptional and well merits record. It was that of a girl, nineteen years old, who had two large vulvar chancres. At the date of evolution of the secondary manifestation she had a mild roseola upon the forehead, lower part of the neck, and of the whole chest. The back was quite well cov- ered, as were also the thighs. She also had pharyngitis. With the evolution of the roseolar eruption the pigmentary syphilide began upon the neck, and within three weeks the whole anterior portion of the trunk was invaded from above downward. In the course of a month involution began around the roseolar spots and also upon the abdomen, where there were no spots. The appearances as shown in the lithographic plate are so clear and striking that further description is not necessary. Here, then, we have a well- marked instance of the coeval appearance of the roseolar and pigmentary syphilide at the very onset of the secondary stage. In six months no evidence of pigmentation could be found on this girl’s body. She also suffered from anal- gesia of the backs of the hands and fingers. This case stands out strongly in refutation of the assertion—based, I think, on faulty and limited observation—that the pigment- ary syphilide always and invariably follows in the wake of some other syphilitic process, exanthematous or papular. From all these facts and considerations 1 am at a loss to understand why two opinions can be held regarding this af- fection which is so decidedly uniform in its development, course, and decline. The third or marmoraceous form of pigmentary syphi- lide is by far the least common. Its mode of invasion is slow and aphlegmasic, and there is little or no hyperpig- mentation. The natural color of the skin, in spots of irregu- lar size and shape, becomes white, while the margins, which are hazy and indefinite, become browner than normal. It seems to be a displacement of pigment resembling strikingly some delicate varieties of marble in which there are imper- ceptibly blended shades of white and very light black. In my experience, this form is always seen on the sides of the neck, and it does not show a tendency to extend. It can only be found upon persons of delicate skin, and very often only by close observation. It slowly disappears and the skin is left in its normal color. As I have already stated, I attach little if any impor- tance to the mass of literature relating to the pathological anatomy of the pigmentary syphilides, since the investiga- tions were made in general at haphazard upon any pig- mented or achromatous skin without any consideration for the stage of the process or for the clearness of the diag- nosis. Maieff’s * observations, made under the direction of Pro- fessor Tarnowsky, are worthy of unqualified acceptance, for the sections of skin were taken only from patients suffering with the primary pigmentary syphilide, and the morbid process was studied upon very many sections made in tis- sues in all the progressive stages from its evolution to invo- lution. Further, these microscopic studies were supple- mented by prolonged and accurate clinical observation. Maieff thinks the pigmentary syphilide is due to a chronic specific inflammation of the minute blood-vessels of the skin which may be due to nutritional changes incident to the early and active period of syphilis. At its inception the morbid process consists in endothelial inflammation with cellular in- filtration into the adventitia of the vessels, which are there- by diminished in caliber and even occluded. As a result of the circulatory disturbance the red blood-cells lose their pig- ment, which escapes and infiltrates the adventitia of the ves- sels, the connective-tissue cells, those of the derma and of the Malpighian layer, and even works its way into the lym- phatics. During the evolution of the process most of the altered vessels become completely obliterated, the papillae become stunted and undergo atrophy. Then the pigmenta- tion begins to be gradually absorbed, the color of the skin grows less intense, and gradually and slowly the discolora- tion disappears, leaving in its wake a whitish surface. These microscopic demonstrations, it will be seen, agree perfectly with the clinical history of the pigmentary syphi- lide and show beyond a doubt that this eruption has a defi- nite and orderly mode of evolution and of involution. In the light of its clinical history and of its pathological anatomy, it is, I think, now clearly proved that this syphi- lide begins as a true specific superpigmentation which is the essential feature of the morbid process, and that the subsequent leucodermatous changes are those of a degenera- tive nature, consequently dependent upon and secondary to the initial dischromia. It can therefore be seen how illogi- cal and incorrect it is to call this affection syphilitic leuco- derma, or syphilitic vitiligo. It is refreshing, when one has gone through a mass of immature and unsatisfactory literature upon this subject by men who have generalized and dogmatized upon feeble and insufficient bases, to read a communication which bears evi- dence of patient, prolonged study by a man who starts in without bias, theory, or prejudice. Such a communication is that on the pigmentary syphilide made bv Dr. Fiveisky * at the suggestion of Professor Pospeloff. (It thus happens that the most reliable and noteworthy of recent contri- butions to this subject have been made at the suggestion of two eminent Russian professors—namely, Tarnowsky and Pospeloff.) After an exhaustive study and research on this subject, Fiveisky has convinced himself that the disease commences with an increased pigmentation of the skin, usually of the neck, and that, after a while, there appear upon the brown surface minute circular or oval white patches or islets, which gradually increase in size and take the place of the diffuse brown pigmentation. This is in direct confirmation of what I have maintained for many years, and which has been denied by several German authors. • There are men who, by their utterances, show that they have observed little and know less concerning this syphilide, * Contribution <\ l’etude de la syphilide pigmentaire. Comptes ren- dm du congrfa international de dermatologie et de syphiiigraphie, Paris, 1890. * Meditzinskoie Obozrenie, No. 2, 1891, p. 167. 6 THE PIGMENTARY SYPHILIDE. who in a flippant way pass it over, or speak of it as a curi- osity unworthy of a place among the numerous manifesta- tions of syphilis. Such a position is both faulty and un- scientific. I have many times been aided in the diagnosis of syphilis by the observation of the pigmentary syphilide when all the other early manifestations had disappeared (even the ganglia in some cases were not sufficiently en- larged to offer aid in diagnosis). So that to me a knowl- edge of the clinical history of the syphilide has been im- portant and helpful. I am therefore glad to see that Fi- veisky says (and his opinion is indorsed by Professor Pos- peloff and Dr. Jebiineff) that this syphilide constitutes one of the most characteristic and most reliable diagnostic signs of condylomatous syphilis (that is, of syphilis in the secondary stage). Before a man makes dogmatic statements and takes a stand hereafter in this question of the pigmentary syphi- lide he must show, first, that he is well versed in der- matology and syphilography in order that we may feel confident of his diagnosis ; second, he must show a full knowledge of the secondary pigmentary and leueodermatous conditions observed in syphilis; third, he must give evi- dence that he has seen at least ten typical cases of this syphilide, and that he has observed and recorded its vary- ing appearances and features from the time of its first in- vasion until its final disappearance; fourth, he should, if possible, study (or have some competent person in histology study for him) the varying pathological changes beginning in the brown stage and running through the course of the disease until its final extinguishment. I hope my readers will insist upon the fulfillment of at least the first three of these requirements. If they will do this, they will see in future fewer of the jejune and misleading essays upon the pigmentary syphilide. For my own part, I like, for the sake of completeness, to append to an essay of the size of this one a complete bibliography, but, though I have it under my hand, in charity I refrain from inflicting it upon my readers. There have been published, however, within the past ten years a few essays of some merit, besides those above quoted, on this subject, but they have not been of such striking character that I deem them worthy of special mention. THE NEW YORK MEDICAL JOURNAL, Edited by FRANK P. FOSTER, M. D. It is the LEADING JOURNAL of America, and contains more reading- matter than any other journal of its class. It is the exponent of the most advanced scientific medical thought. Its contributors are among the most learned medical men of this country. Its “ Original Articles ” are the results of scientific observation and research, and are of infinite practical value to the general practitioner. The “Reports on the Progress of Medicine,” which are published from time to time, contain the most recent discoveries in the various departments of medicine, and are written by practitioners especially qualified for the purpose. The Society Proceedings, of which each number contains one or more, are reports of the practical experience of prominent physicians who thus give to the profession the results of certain modes of treatment in given cases. The Editorial Columns are controlled only by the desire to promote the welfare, honor, and advancement of the science of medicine, as viewed from a standpoint looking to the best interests of the profession. Nothing is admitted to its columns that has not some bearing on medicine, or is not possessed of some practical value. It is published solely in the interests of medicine, and for the upholding of the elevated position occupied by the profession of America. The volumes begin with January and July of each year. Sub- scriptions must be arranged to expire with the volume. SUBSCRIPTION PRICE, $5.00 PER ANNUM. D. APPLETON & CO., Publishers, New York, Boston, Chicago, Atlanta, San Francisco*