Raynaud’s Disease. BY J. C. SHAW, M.D., Clinical Professor of Nervous Diseases at the Long Island College Hospital. Brooklyn ; Medical Superintendent of the Kings County Insane Asylum. REPRINTED FROM 2Tlie 'Neto ¥orh ftteDical Journal for December 18, 1886. Reprinted from the New YorTc Medical Journal for December 18, 1886. BAYNATID’S DISEASE* By J. C. SHAW, M. D., CLINICAL PROFESSOR OF NERVOUS DISEASES AT THE LONG ISLAND COLLEGE HOSPITAL, BROOKLYN ; MEDICAL SUPERINTENDENT OF THE KINGS COUNTY INSANE ASYLUM. The condition to which I invite your attention is one of considerable interest, and only within recent years has the attention of physicians been directed to it. For the purpose of elucidating the clinical history of this condition, I shall give briefly the histories and descrip- tion of some cases. The marked features in these cases are the localized blanching of the fingers or toes, nose, or ears, and in some cases localized gangrene of these parts: Case I (Personal).—A woman, aged fifty, admitted to the asylum with melancholia, delusions of poisoning, etc., attempts at injury to herself, refusal of food, and resistance to every effort made to do anything for her. One morning when visit- ing her I picked up her band to examine her pulse, and was surprised to find the index and middle fingers, up to the joint of the second phalanx, extremely pallid and death-like. When felt, they were intensely cold. This condition of pallor lasted some time, then passed away, and I never observed it again in the patient. It was impossible to learn if this condition had ever occurred before to her. * Read before the Brooklyn Pathological Society, April 22, 1886, 2 RAYNAUD’S DISEASE. Case II (Personal).—M. S., aged fifty-one, seen first Decem- ber 12,1882, having been sent to my clinic for nervous diseases, in the Brooklyn Eye and Ear Hospital, by Dr. Alexander Clark. Patient is of spare build, but healtby-looking. lias enjoyed good health in former years. Eight months ago began to have some trouble with right shoulder; could not move it as well as formerly, and, when she did move it to any great extent, it pained her. About this time she began to notice that the little and ring fingers of the left hand would suddenly become cold and white, lasting about half an hour, and then pass away. At first she had these attacks about once a week; at present she has them every day. AVliile being examined she had an attack. The entire little finger, and the next finger up to the joint of the second phalanx, became suddenly intensely pale and death-like; they were cold to the touch, and the patient said they felt cold. This pallor lasted about half an hour; it was followed by a feeling of warmth, and, as the patient said, one could almost see the blood as it returned to the fingers; they grew red and warm. Case III (Dr. T. A. McBride, in a paper before the New York Neurological Society, April 1, 1878; partially reported in New York “Medical Record,” April 11, 1878).—Man, aged thirty- two, of neurotic constitution. Certain parts of the index and middle fingers of both hands, also of end of ring finger of right hand, at irregular intervals and at varying periods, became cold, of a dead-white paleness, numb, and stiff, with some loss of sen- sibility, and were moderately painful. These symptoms were replaced by a dusky, often purplish-red color of the same parts, accompanied by a sensation as of having been stung by nettles, and soon after the affected portion of the fingers re- gained their normal appearance. The phenomena appeared to occur independently, occurring when the patient was otherwise healthy as well as when sick. No similar phenomena had been observed elsewhere in the body. Case IV (Dr. Barlow, “ Clinical Society Transactions,” April 27, 1883).—In October, 1882, there appeared in the out-patient room a little girl aged five. Within a short time the whole of right foot and ankle, and the leg for a distance of three or four RAYNAUD’S DISEASE. 3 inches upward, had become cold and of a nearly uniform gray- ish-blue color. The child whimpered a little with pain in the foot, and did not like it to be handled much. Just below both elbows, on the dorsal surface of the forearm, there was an area of perhaps three inches in length, of ill-defined blueness and coldness. This slight blue area was more marked on the right than on the left side. The child had no cardiac disease. It was admitted into the hospital ward of Dr. Fox, and this con- dition passed off in an hour or two, and the next day the child seemed perfectly well. The second day after, at 3.30 p. m., while sitting in the ward, she had another attack, which Dr. Barlow saw. There was slight blueness and coldness in the left foot and ankle. The child whimpered a little, but was able to walk across the ward with a slight limp; the foot became natural again within less than half an hour, and she had no further trouble. From a his- tory obtained subsequently, it was learned that she was nursed nearly two years, was late in teething, and was probably rickety to some extent. She had not suffered from any of the acute specific diseases, and there had been nothing special in her his- tory until February, 1881, when she was about three years and a half old. It was very cold weather, and the child was out of doors, but carried in somebody’s arms. When brought into the house she complained of cold feet, and vomited some watery stuff. Her feet were quite blue, and the blueness extended for a short distance above the ankles. The blueness lasted from five o’clock until midnight. Next day she was all right. Seven months passed before she had another attack, and this was in the month of September. The blueness lasted two hours. She had another attack in a week’s time, and several other attacks during the winter. Since that time the attacks have alwmys been confined to the cold weather. The attacks occur mostly in the afternoons; never at night. The duration of the longest attack has been seven hours; but usually the attacks pass off in less than an hour. Latterly the elbows have been affected, but the ears and nose never have been. Case V (Dr. Barlow, “ Clinical Society Transactions,” 1883). —A girl, aged five, of healthy parentage, had been nursed twelve 4 RAYNAUD’S DISEASE. months, and, with the exception of what was called “ congestion of the brain from teething ” at sixteen mouths, had been a healthy child. She had had whooping-cough and measles when one year old ; never had ague. In September, 1881, being then three years and a half old, she had her first attack of coldness and blueness, with pain affecting one foot, and lasting for several hours. Very soon after this attack commenced, the child passed some very dark urine. She had another similar attack in a few days, and then repeated attacks during the winter until April, 1882. There were sometimes two or three during the week; the dura- tion was not generally more than three hours. The attacks occurred most commonly at midday; the pain always pre- ceded the coldness and blueness. The child did not pass dark urine with every attack, and never more than once with each attack. During the first winter, along with these symptoms, she complained on some occasions of pain in her stomach, and in one attack the left hand became blue and cold up to the wrists. The last attack of the first winter was on May Gth. She was then free until September, 1882. Dr. Bar- low saw her in February, 1888. Up to that time she had some- times gone fourteen days without an attack, and at other times she had had two in a day. March 15th.—For about eight days she has had an attack daily in left foot, but on some days in both. Case VI (Dr. T. C. Fox, “Clinical Society Transactions,” 1885).—Mrs. S., aged forty-one; family history good; no evi- dence of any neurosis ; never had rheumatism or any special ill- ness; married at twenty-seven, and had six children, including twins at last confinement; no miscarriages. Dates her illness from June, ten years ago, when she moved into a damp house. Her fingers then began to grow at frequent intervals white and dead, all of them simultaneously on both hands, “like wax,” as she expressed it, and they pained her excessively. Gradually her feet also became involved, and up to the present time the recurrences have been getting more frequent. She is now a woman of spare habit, aud with an anxious face and intensely nervous temperament. She sleeps poorly, and has had two attacks of hysteria. Heart and other organs healthy. There is RAYNAUD’S DISEASE. 5 no diabetes. When seen in February the condition of the fin- gers might easily have been mistaken for scleroderma; all of the fingers were icy cold, of a slightly blue tinge, fusiform in shape, the skin over them shining and sunken; the nails were variously affected, and some of the bones atrophied. The his- tory, however, made the nature of the malady clear. On sub- sequent occasions I have seen all the fingers slightly swollen and of various shades of lividity up to the metacarpo-phalan- geal joints. Occasionally the asphyxiated condition of one of the fingers will lead to the formation of a blood-blister; the contents subsequently become puriform, and, on the rupture of the bullae, an ulcerated surface is left, which is very slow to heal. At the time of writing, the left ring finger is ulcerated at the end and under the nail. In the toes the disease is less severely felt, blit their ends are somewhat blue and cold. The tip of the nose is rather blue. The influence of cold is very marked. Her ears and nose grow blue and cold directly she goes out of doors; her hands and feet get intensely livid, with an “agony of pain.” She also states that her extremities grow blue “ in a second ” if she is startled by a sudden knock at the door or any unusual occurrence, and her heart “ feels as if in a vise.” Her pulse is thready and compressible. There has been no hsemoglobinuria, and her blood appears to be normal. Case VII (Dr. T. 0. Fox).—Man, aged fifty-one, of regular life. He was a stout, florid-complexioned man of remarkably good physique, but yellow conjunctiva; complained of feeling weak. He applied to Dr. Fox on account of two oval, sym- metrical, gangrenous sores, of about the size of a half-crown, one at the junction of the middle and lower thirds of each shin. These sores were covered with an adherent black eschar, and they had originated suddenly in blood-blisters. There were also a few pustules scattered around. His feet and hands were cold and his pulse was soft and weak, but, with one exception, Dr. Fox could find no other diseased condition. He was suffering from marked diabetes, but was not aware of it. The sores were very obstinate to heal, and on March 28th another congestive patch appeared on right shin, which seemed to threaten gan- grene. On January 2, 1884, he again applied to Dr. Fox, who 6 RAYNAUD’S DISEASE. says he did not recognize that the man was suffering from Ray- naud’s disease until January 5th, when his left great toe became suddenly black and swollen and intensely painful. Inquiry now developed the fact that for years he had suffered from sudden blanching and numbness of the digits. He had formerly been a door-porter in a store, and exposed to draughts. Case VIII (Dr. Charles K. Mills, “American Journal of the Med. Sciences,” 1878, p. 431).—E. J., aged thirty-two, single, mill-hand. Mother died of phthisis; well until nineteen years old, except always suffering from excessive sweating of hands and feet. At nineteen she took a severe cold, from which she suffered several weeks. One morning in the early autumn, on her way to work, the little finger of her left hand, as far as the second joint, suddenly became cold, white, and numb. After this, whenever exposed to cold, one or more fingers would pre- sent this appearance. Dr. Mills found a mitral presystolic murmur; the lungs showed impaired percussion resonance and vesicular murmur, and there was a cavity of considerable size in the right apex. Six months after her first attack her little finger was first affected. A swelling formed at the point of the thumb as if a splinter were in it; it suppurated and a small ab- scess formed, and was a long time in healing; from time to time thereafter small abscesses formed on ends of fingers. Case IX (Personal).—M. D., aged forty, an insane woman, confined in the Kings County Insane Asylum. Patient has always been of a disagreeable, fault-finding disposition, and of a very low order of intelligence, which her physiognomy also in- dicates. She has a paternal aunt insane. Patient has had de- lusions of poisoning, with consequent refusal of food ; made many attempts at suicide ; has often been violent, owing to her delusions of persecution. In August, 1883, she became quite agitated and remained in this condition many months, talking in a loud voice, attacking all who came near her; in constant motion; never sat down. In September it was observed that she had a small gangrenous spot on the end of the middle finger of the left hand ; she resisted when an attempt was made to look at it, but was held; it wras then found that she had complete gan- grene of the end of that finger, and each finger on that hand had, RAYNAUD’S DISEASE. 7 on each side of the nail, a small black gangrenous spot. The gan- grene of the first phalanx of the middle finger in the next week extended so as to involve the entire phalanx; it "was black, and evidently quite dead and sloughing. The small spots on the other fingers had grown a little larger. It was now found that the right hand had also begun to have the same small black spots on every finger on each side of the nail; the gangrenous spots on this hand were not larger than the head of a large pin. Her feet were examined, but no similar condition was found, al- though they were very much swollen. Her urine showed noth- ing abnormal. Her heart could not be examined, as she had to be held and fought constantly when approached, but the record shows that, on her admission, her heart was normal. In the course of several weeks the black spots on the right hand dis- appeared without ulceration, but on the left hand the middle finger ulcerated and sloughing took place. At the present time the finger is not yet well; the ulcerated surface is still not healed; she will not allow any dressing to remain on it. Case X (Dr. Southey, “Clinical Soc. Trans.,” 1883).—F. N., aged nine, a large-headed, fair-complexioned, light-haired child. He enjoyed good health until the autumn of 1881, when he was attacked with some feverish illness, attended by pain in his limbs, which was believed to be rheumatism. On admission, he was greatly emaciated, and his hair was falling off in large quantities. He was in a very nervous and excitable condition, crying whenever an attempt was made to examine him, singing snatches of songs and hymns in a loud tone; by day he slept a good deal, but as evening approached he became very noisy, singing and screaming alternately. He used to pull handfuls of hair off his head. Appetite bad; tongue clean and moist; pulse, 148; no cardiac murmur, no physical signs of lung dis- ease ; urine presented no abnormality. The single noticeable fact observed was the localized gangrene of the tip of his right index finger and the coldness of his extremities. The gangrene of the right forefinger crept slowly and steadily onward, and, in a few days, involved the entire terminal phalanx. On De- cember 2d the thumb and second finger of the right hand were 8 RAYNAUD’S DISEASE. observed to be red, swollen, throbbing, and hot, just like chil- blains, and there was a small purple patch upon the helix of his left ear, evidently a blood stasis. On December 4th this patch was still apparent, although smaller, but the third tinger was assailed like the thumb, fore, and second fingers, and all four looked extremely livid and red, while redness and swelling of the thumb of the left hand were also present. On December 5th a spot exactly similar to that upon his ear of local blood stasis, threatening gangrene, appeared upon the extreme tip of his nose, and the tip of the right middle finger became quite black. During the ten days that the boy had been under observation his temperature was found in the morn- ing, before 10 a. m., to be usually normal or only slightly ele- vated, 99-4°; in the afternoons and evenings it rose to from 101° to 102*8°. On December 19th the tips of all the fingers of his right hand were gangrenous, and the fiesli of the fingers below the gangrened ends was livid and swollen. On the left hand, the thumb, index, and little fingers were extremely dusky. Tn the course of the ensuing week the gangrene made rapid progress, spreading from the lips of all the fingers of the right hand backward and centripetally. On December 29th there were troublesome cough, broncho-pneumonic sputum, and tubu- lar breathing over the lungs posteriorly. In the first week of January his general condition was improved; lines of demarka- tion between dead and living tissue were defined. Suppuration commenced. The cough improved, and he began to gain a little flesh. On January 9th the urine was found to be acid, D012 spe- cific gravity, and containing both blood-cells and albumin. It attracted attention by its dark color. For several subsequent days it was natural-colored and of specific gravity varying between 1‘010 and 1-025. He presented a true intermittent haematuria, provoked apparently by impres- sions of external cold on the surface of his body. Thus, after exposure and being washed, he would pass a specimen of urine RAYNAUD’S DISEASE. 9 containing blood, whereas urine passed a few hours later con- tained a mere trace or none. During the latter part of January, February, and March, blood was more often absent than present in the urine, and this only very temporarily. Throughout January suppuration and separation of the gan- grened digits was taking place. Ilis temperature ranged between OT-f0 and 101‘4°; it always rose in the afternoon and sank in the morning. At the begin- ning of August he was able to walk about again. Most of these patients presented an evident abnormal ir- ritability of the nervous system, if not positive nervous dis- order. Dr. McBride’s patient had anterior spinal paralysis, from which he recovered. One of Dr. Barlow’s patients was said to have had “ con- gestion of the brain from teething.” One of Dr. Fox’s patients eventually became a nervous hysterical woman. Dr. Southey’s patient was evidently hydrocephalic, and probably insane, when under his observation. One of Dr. Fox’s patients had diabetes with an evident neuropathic constitution to begin with. My three cases presented a neuropathic history. The exciting causes of the disease appear to be exposure to cold, most of tbe cases being very much worse in the cold weather. Emotional disturbances, fright, etc., appear also to be excitants. Many of the cases are associated with intermittent ha3- maturia, which is probably due to the same causes as the gangrene and “ dead fingers.” Some of the cases are also associated with diabetes. The symmetrical distribution of the gangrene, and also 10 raYNaud's Disease. of the blanched fingers, is remarkable, and was especially insisted upon by Raynaud. Although a large number of the cases are symmetrical, especially those leading to gangrene, many are reported in which only one extremity is involved. This symmetry in the attack is seen even in cases in which the ears are the seat of the gangrene. According to McBride, the development of dry gan- grene from digiti mortui is rare. According to Raynaud, the condition usually preceded what he called local asphyxia, which might be acute or chronic. If acute, it might go on without any disturbance of nutrition, but more commonly gangrene, more or less localized, occurred, and sloughs of considerable size might form, or, what seemed more common, after a small amount of gangrene had occurred, the local asphyxia disappeared and cicatrization followed. In the chronic form, lasting some months, there might be localized gangrene followed by cicatrization, as in the acute, but more commonly there were manifested other alterations in the nutrition of the parts, so that the skin became much thickened and indurated. Dr. Colcott Fox thinks that the changes left after this disease may lead to its being confounded with scleroderma. It can, however, only be so confounded if a previous history is not obtained. Dr. Fox also remarks that in two unques- tionable cases of scleroderma under his observation, in which the hands were involved, both women had long been subject to “ dead fingers,” and one of them continued to have mild attacks of asphyxia of the fingers after the onset of scleroderma. It appears also that Vidal thinks there is an intimate connection between scleroderma and Raynaud’s disease, and ftAYNAtlD’S DISEASE. 11 Brochin points out that the two diseases can be united in the same patient. Dr. Allan McLane Hamilton, in an article on this sub- ject, sums up by saying that it is characterized by— 1. Local blanching, lasting two or three weeks, or com- ing on in intervals of several weeks and lasting a few hours each time. 2. Sensation and motion unimpaired. 3. Temperature of affected members lowered. 4. Parts affected—hands. Subsequently reported cases, however, have shown that we may have slightly elevated temperature as well as low- ered, and the hands, feet, ears, nose, and even the body, may be the seat of these disorders. A marked characteristic of these symmetrical gangrenes of the extremities is the complete absence of all causes of obstruction in the arteries. How, then, is this interruption to the flow of blood into the parts to be explained ? Ray- naud thinks that it is the result of a spasm of the small blood-vessels under the influence of an irritation to the vaso-motors of these vessels, and usually through a reflex act. Raynaud thought that, in women who were the subjects of this disease, the pelvic organs might be the starting- point of the irritation, which was reflected upon the vaso- motors of one or more of the extremities, for some cases of localized asphyxia were observed at the time of menstrua- tion. Many cases, however, appear to have had their pri- mary seat of irritation in the extremities themselves—such cases as were produced by cold, for instance. It appears probable that, whenever the vascular disor- ders are symmetrical or wide-spread, the monarchical vaso- motor center in the medulla oblongata may be involved, and one might be inclined to think that in these cases occurring 12 RAYNAUD’S DISEASE. in association with diabetes, this monarchical vaso-motor center was involved, and thus caused the localized spasms at the extremities, etc. Dr. T. A. McBride also explains the condition as being due to reflex vaso-motor spasm. Vulpian does not fully accept Raynaud’s view as to menstruation producing the starting-point from which reflexly the vaso-motors of an ex- tremity are affected; he does not think this necessary to ex- plain these cases, for he says at the menstrual period, in many persons, the nervous system is already often in a state of over- excitability ; the least impression on the skin is apt to give rise to phenomena out of all proportion to the excitation. More recently Raynaud has expressed the view that in these cases there is an exaggeration of the excito-motor power of the central parts of the spinal cord presiding over the vascular innervation. Dr. Hamilton also looked upon this condition as due to over-irritation of local sympathetic vaso-motor filaments. One who has the occasion to watch the approach of the blanching of the fingers in one of these cases at once adopts the view of its being due to vaso-motor spasm. I have been asked to point out the treatment to be adopted in such cases. I must do so in the most general manner possible; it has been various, almost every writer adopting a different plan. Galvanism, locally, also to the spine, has been quite gen- erally used. Raynaud first advised it. Bromide of potassium, tonics in general, Fowler’s solution, potassium iodide, quinine, morphine, internally, when the patient has suffered pain, and the local application of chloroform, have also been used for the same purpose; light bandaging of the part has also ap- peared to relieve this pain. In cases complicated with dia- betes, scleroderma, etc., the treatment indicated is such as is carried out in those conditions. RAYNAUD’S DISEASE. 13 Other than those cases of Dr. Allan McLane Hamilton, Dr. T. A. McBride, and Dr. C. K. Mills, a case has been reported by Dr. S. C. Clark, of Clayton, N. Y. (“Med. Record,” 1885, vol. i, p. 122). Dr. C. L. Dana, in an article on “ Aero-Neurosis,” re- ports two cases (“Med. Record,” 1885, vol. ii, p. 57). Dr. Dana, at the end of his article, gives an extended bibliog- raphy, and to that I refer those interested for cases other than those mentioned in this brief essay. REASONS WIIY Physicians should Snteilie - FOR - The New York Medical Journal, Edited by FRANK P. FOSTER, M.D., Published by D. APPLETON & CO., 1, 3, & 5 Bond St. X. BECAUSE : It is the LEADING JOURNAL of America, and contains more reading-matter than any other journal of its class. 2. BECAUSE : It is the exponent of the most advanced scientific medical thought. 3. BECAUSE : Its contributors are among the most learned medi- cal men of this country. 4. BECAUSE: Its “Original Articles” are the results of sci- entific observation and research, and are of infinite practical value to the general practitioner. 5. 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