Part 79

Those who survive the onset of malignant scarlet fever often have in the course of a few days severe pharyngitis, with extension of the inflammation to the lymphatic glands and connective tissue around the angle of the jaw. These inflammations cause more or less external swelling. The faucial turgescence around the entrance of the larynx, with the accompanying secretion of viscid mucus or muco-pus, often causes noisy respiration, and many at this stage of the attack breathe with the mouth constantly open to facilitate the ingress of air.

Ordinarily, no discharge occurs at first from the nasal surface, but as the disease continues, if the type remain severe, defluxion of thin muco-pus takes place from the Schneiderian surface, which frequently excoriates the cheek. The lips also are apt to be sore and swollen.

In malignant cases the disease is more protracted than when the type is mild. Thus in a recent case in my practice the rash was still distinct at the close of the second week, though the temperature had fallen from 105° to 102° and some desquamation had appeared. Long continuance of the febrile movement is, however, oftener attributable to some inflammatory complication than to the primary disease.

In all epidemics of a severe type cases now and then occur in which the poison is so intense, or it acts with such frightful energy, that death occurs even within the first day. The patient is overpowered at the outset of the disease by the virulence of the specific principle, perishing in coma, preceded perhaps by convulsions. The autopsy in such cases reveals hyperæmia of the brain and cranial sinuses, blood of a dark-red color, capillary hemorrhages in various parts, a flabby heart, and perhaps some engorgement of the spleen and kidneys.

Usually, malignant scarlet fever exhibits its severe type from the first, but cases sometimes occur which seem mild and favorable for a few days, when severe symptoms suddenly supervene. This change from a mild to a dangerous disease is, however, most frequently, I think, due to some complication.

IRREGULAR FORMS.--Deviation from the normal type in scarlet fever is usually due to some perturbating cause, which is often a pre-existing or co-existing disease, or a disordered state of system through causes distinct from the scarlatinous disease. Thus, a little girl in my practice had the symptoms of scarlet fever, such as febrile movement and inflammation of the buccal and faucial surfaces, nearly a week before the scarlatinous eruption appeared. During this time the patient had an intestinal catarrh, with diarrhoea, which declined when the rash occurred. This intestinal disease was the apparent cause of the irregularity in the malady. If scarlatina occur during a severe attack of entero-colitis attended by purging, the defluxion from the external surface may be such that no efflorescence appears. Severe scarlet fever itself sometimes appears to cause gastro-intestinal catarrh so as to produce an afflux of blood toward the intestinal tract and away from the skin. Practitioners occasionally meet cases like the following, which I recall to mind: In a family where scarlatina was prevailing a little child early after the commencement of symptoms which seemed to be plainly referable to this exanthem was seized with vomiting and purging, which continued till death {509} occurred on the third day. No efflorescence appeared upon the skin, but the symptoms indicated the presence of severe intestinal catarrh, complicating and masking scarlatina. We are aided in the diagnosis of such cases by observing the faucial redness, and we may discover a faint efflorescence upon parts of the surface, as about the groin or in the flexures of the joints. In another instance an infant in the warm months having protracted entero-colitis, the usual summer epidemic of the cities, had the characteristic symptoms of scarlet fever, which was present in the family, but the diarrhoea continued and no rash appeared.

In one who is much reduced by an antecedent disease, as phthisis, or who has a disease, chronic or acute, which produces a decided afflux of blood away from the surface and toward the interior of the body, the eruption is commonly tardy in its appearance, indistinct, or wholly absent. Thus, severe inflammations of internal organs not infrequently render scarlet fever irregular. On the other hand, some maladies occurring in connection with this exanthem do not change its symptoms, but themselves undergo modification. Pertussis may be cited as an example, the cough of which is sometimes modified by an intercurrent attack of scarlet fever, the symptoms of the latter disease undergoing little change.

Scarlet fever may also be irregular without any apparent perturbating cause. In 1867 I attended a young lady whose previous health had been good, and whose brother was sick at the time with scarlet fever. She had considerable febrile movement, with severe pharyngitis, and, though her surface was repeatedly examined, no efflorescence was seen. Two weeks subsequently she was affected with severe nephritis, anasarca, effusion into at least one of the pleural cavities, oedema of the lungs, and probably hydro-pericardium, the case ending fatally. Rilliet and Barthez state that a second attack of scarlet fever is more apt to be irregular than the first. Probably this opinion is correct, especially if only a short time have elapsed between the two seizures. Still, as we have already stated, both seizures may be typical, and the second more severe than the first.

It would be impossible to make a clear and positive diagnosis of certain cases of irregular scarlet fever, in which cerebral, pulmonary, or gastro-intestinal symptoms predominate, were it not for the fact that they occur in connection with other cases of scarlet fever or are followed by sequelæ which evidently have a scarlatinous origin.

Occasionally, the eruption, if it be intense or if a certain condition of system be present in the patient, is accompanied by more or less extravasation of blood-corpuscles from the capillaries, so that the redness does not entirely disappear on pressure, usually in points. In rare instances certain of the exanthematic fevers present an extreme hemorrhagic character, so as to be beyond the reach of remedies, and of necessity speedily fatal. Hemorrhagic cases of this severe form are probably more common in variola than in the other fevers, but I have met a notable case in what was diagnosticated scarlatina. In June, 1881, a man in his thirty-second year, whose previous health had not been good, though he had no defined ailment and had been able to follow his occupation of harness-maker, suddenly became very ill, with high febrile movement and faucial inflammation, attended by marked prostration. After some hours an intense eruption of a scarlatinous appearance covered nearly the entire surface, and on the following day hemorrhages began to occur. The urine {510} contained a large proportion of blood; each conjunctiva was raised by hemorrhages underneath (ecchymosis), so that its natural color was lost and the eyelids closed with difficulty; and blood flowed from the nostrils, gums, and under the skin, forming hemorrhagic points and blotches. One of the consulting physicians, perceiving the resemblance to hemorrhagic variola as described by Hebra, suspected that we had a case of this formidable malady to deal with, but the time for the appearance of the variolous eruption passed by without its occurrence. Death took place on the fifth day. The temperature during the sickness was high, though the record of it has been mislaid. Fortunately, such severe hemorrhagic cases, which are necessarily fatal, are rare.

COMPLICATIONS AND SEQUELÆ.--Scarlet fever, if its type be severe, is in itself dangerous to life. Many, as we have seen, perish from its direct effects when it produces profound blood-poisoning. But, while the ordinary epidemics of this malady are necessarily attended by a large mortality from the virulence and depressing effect of the specific principle, unfortunately, of all the diseases of modern times, scarlatina ranks first as regards the number and gravity of its complications and sequelæ, so that nearly or quite as many perish from these as from the direct effect of the poison.

Nervous accidents occur chiefly at two periods--to wit, in the first days, when they are due to the severity and malignancy of the malady and to the impressible nervous temperament of the child, and in the declining stage, or after the termination of the fever, when they occur from uræmia. If the type be malignant, delirium, jactitation, profound stupor, and convulsions frequently occur on the first and second days; and they are symptoms which properly excite the utmost alarm and demand all the resources of our art, since they indicate a form of the disease which is apt to end in speedy death. The eyes have a dull or wild expression, the conjunctiva is suffused, the heat of surface pungent, the pulse rapid and compressible or feeble, rising above 150, even to 200, per minute, and the temperature is always elevated to a degree that involves danger, the thermometer not infrequently indicating 105° or 106°. But this severe form of scarlet fever, attended by so great elevation of temperature, is much less dangerous than in former times, even though it be complicated by delirium and convulsions, since we no longer hesitate to reduce bodily heat, when excessive, by the free use of cold baths, and have discovered potent agents in the bromides and chloral for controlling convulsions. Nevertheless, not a few perish in the commencement of scarlet fever with predominating cerebral symptoms, as delirium or eclampsia, followed by coma, under the best possible treatment. Sometimes the symptoms have closely simulated those of acute meningitis, and if the rash have been delayed and the sore throat is as yet slight, the physician may suspect that he is dealing with this disease; but autopsies in such cases show no inflammatory lesions, but only congestion of the cerebral and meningeal vessels.

As is stated in a preceding page, in every case of normal scarlet fever inflammation of the faucial surface is present, as indicated by redness, tenderness, and increased secretion of mucus or muco-pus. It precedes the efflorescence on the skin, and is announced by pain in swallowing and on pressure with the fingers behind and below the angles of the jaw. In that form of scarlet fever which has been designated anginose the {511} pharyngitis is severe, and is a prominent element in the malady, the uvula, the pillars of the fauces, and the faucial surface in general being infiltrated and swollen. Nevertheless, this inflammation, with the accompanying tumefaction, is properly a part of the disease, rather than a complication, if it abates with the subsidence of the scarlet fever or begin to abate soon after, and if it produce but slight destructive change in the tissues of the neck. The secretions from the fauces may be foul and offensive; even superficial ulcerations or gangrene may occur upon the faucial surface, causing it to present a dark brown or jagged appearance, and the tissues of the neck may be infiltrated to a certain extent, and we designate the disease a form of scarlet fever under the title anginose. But when this condition is greatly aggravated, so that there is extensive infiltration and swelling of the tissues of the neck, with an amount of ulceration or gangrene which in itself involves danger, continuing after the primary disease abates, prolonging the fever and reducing the strength, it is proper to regard the state of the throat as a complication. In addition to the pharyngitis, which is severe as described above, the sides of the neck around the angles of the jaw become swollen, hard, and tender. The inflammation has been propagated to the deeper structures of the neck. Poisonous substances, the result of decomposition or vitiated secretions, traverse the lymphatic vessels from the faucial surface, and, being intercepted in the lymphatic glands, cause adenitis, and the inflammation extends from the glands to the adjacent connective tissue, which becomes hard, tender, swollen, and infiltrated with inflammatory products. This tumefaction sometimes begins by the second or third day, but it is usually about the close of the first week or in the beginning of the second week that it becomes so considerable as to constitute a source of danger and anxiety. It is in most cases bilateral, though one side may begin to swell before the other and remain larger throughout.

In severe cases of this complication the tumefaction extends from ear to ear, filling up the space below and around the angles of the jaw and under the chin. Not only is deglutition difficult, but it is difficult to open the mouth sufficiently to inspect the fauces, and attempts to do so cause much pain. The lymphatic glands, which lie in the inflamed area and participate in the inflammation, are greatly enlarged by hyperplasia, the round granular lymph-cells multiplying so abundantly that the glands increase to many times their normal size. Most of the tumefaction is, however, due to extension of the inflammation to the connective tissue of the neck. The cellulitis, which resembles that occurring in other conditions, is attended by distension of the capillaries, the abundant formation of young round cells, and transudation of serum (Billroth). A moderate amount of tumefaction may disappear by resolution, but if it be considerable it seldom abates in this way, but by the tedious and exhausting process of suppuration or gangrene. If the swelling at its most prominent point present a reddish hue, all hope of producing resolution must be abandoned; it cannot be effected by any medicine or appliance within the resources of our art. The abscess which forms is apt to be diffuse, so as to involve danger of pyæmia, unless it be soon opened and properly washed out. With the discharge of the pus the swelling gradually softens and declines. In other cases gangrene results. The vessels in the inflamed part are compressed by the inflammatory products, so that {512} they no longer convey the blood which is required for the purpose of nutrition. It is a law of the economy that whenever the circulation ceases, the tissues which receive their nutritive supply through the obstructed vessels lose their vitality. Hence gangrene occurs in all that portion of the swelling in which the circulation is arrested. The skin over it peels off, the dead tissue underneath is brown or dark, and soon, if life be prolonged, the slough begins to separate. The prognosis as regards this complication depends largely on the size of the slough. If it be large, death will probably result, since the strength of the system is already reduced by the primary disease, and the reparative process will necessarily be slow, while abundant suppuration tends to increase the exhaustion. In some of the worst cases of cervical gangrene which I have seen the slough has laid bare the muscles and vessels of the neck, producing in one case a cavity or excavation sufficiently large to admit a hen's egg. Often the slough extends under the skin, so that the deepest recesses of the cavity are not visible, and occasionally in cases which have ended fatally in my practice severe hemorrhage occurred from the concealed vessels. If the ulcerative or gangrenous process extends so deeply into the tissues of the neck that hemorrhages occur, death is the common result; but if the destructive action be of moderate extent and other conditions favorable, we may expect recovery through cicatrization, with perhaps some deformity by contraction of the cicatrix.

When the inflammation of the connective tissue of the neck is extensive, involving both the lateral and anterior regions of the neck, the patient is in a perilous state. The cellulitis, when extensive and accompanied by much swelling, may produce oedema of the glottis, may obstruct respiration by compressing the air-passages or the laryngeal nerves, may cause compression of the jugular veins, and thus give rise to dangerous cerebral symptoms, or may lay bare and injure important muscles and nerves, as we have seen. If the ulceration or gangrene be extensive, and death do not occur by hemorrhage from arterial or venous twigs, septic poisoning may occur, increasing still more the fatal nature of the malady.

Some cases of this complication are melancholy in the extreme, as one related by Cremen, in which ulceration of the pharynx occurred, allowing the escape of food and preventing deglutition. In severe scarlatinous pharyngitis the inflammation is apt to extend along the Eustachian tube, causing its occlusion. This accident will be considered when we treat of otitis media, another grave complication. It often also extends into the nares, causing catarrh of the Schneiderian mucous membrane, with discharge of muco-pus from this surface. Not infrequently ulceration or gangrene occurs in the faucial surface, producing more or less destruction of tissue and forming excavations which connect with the throat, while the cutaneous surface retains its integrity and is not even reddened. The following case shows how grave the complication which we are now considering sometimes is when the external surface of the neck is not involved, and how the inflammation by extension outward from the fauces may involve the middle ear.

Case 1.--Annie K----, aged two and a half years, an inmate of the New York Foundling Asylum, was well, except an eczema of the scalp, until the night of April 3, 1882, when she was attacked with vomiting and {513} diarrhoea. She was feverish and drowsy, and at 2 P.M. on the 4th the scarlatinous efflorescence appeared upon her neck, body, and lower extremities; tongue coated; pharynx red; temperature (axillary) 103°; pulse 160. The symptoms and aspect indicated a grave form of the malady, and the usual sustaining treatment was ordered. On April 5th the temperature was 102°, pulse 144, tongue less coated, eruption fading, less stupor, no albumen in urine. April 6th, morning temperature 102°, pulse 160; passed a restless night; stools thin and too frequent; has grayish patches in the throat: P.M. temperature 103-1/5°, pulse 150. April 7th, the diarrhoea continues, and she has a copious muco-purulent discharge from the nostrils; P.M. temperature 103-3/5°, pulse 160. April 10th, the temperature has continued at about 103°; the patient is very sick, with a constant foul-smelling discharge from the nostrils; breath very offensive; temperature 103.5°, pulse about 180. April 12th, general appearance a little better, but the posterior surface of the fauces is completely covered by a thick pseudo-membrane; had four loose stools last night; temperature and pulse the same as at last record; a dark, offensive, and jagged coating over the fauces, and a dark, foul discharge from the nostrils, as before; examination of the chest negative. April 14th, is much prostrated; temperature 104.5°, pulse rapid and weak; respiration noisy, diminished resonance over lower two-thirds of left side of chest; ulcers upon the mouth and tongue; fauces red and ulcerated. April 17th, pulse 150, temperature 100.5°; general appearance somewhat better, but the diarrhoea continues, and patches of a diphtheritic character have appeared upon the lips; moist râles in left side of chest. The symptoms continued nearly the same until April 23d, when she died. A dull percussion sound and distinct bronchial respiration were observed in the left scapular region during the last days of her life.

Autopsy nine hours after death by the curator, Dr. W. P. Northrup: Body well nourished; the tissues have a jaundiced hue; lips sore; on turning the head to one side pus runs from the left ear and dirty muco-pus from the mouth. Brain normal; on opening the petrous portion of the left temporal bone the middle ear is found full of pus, which communicated freely with the external ear through a perforated membrana tympani; the Eustachian tube cannot be traced in the sloughy tissue, and a passage filled with pus extends from the ear to the fauces; opposite the greater cornua of the hyoid bone are two deep ulcers, each having about the diameter of a ten-cent piece, with sloughy and offensive base and sides; the left ulcer communicates by a ragged and wide sinus with a dark and sloughy cavity of about four drachms capacity; this cavity is located in the neck under the angle of the jaw, apparently occupying the site of a disintegrated gland, and it opens upon the surface of the fauces. The surface of the larynx has a dusky, dirty appearance, sprinkled with little cheesy-looking spots, and covered by a dirty, foul-appearing liquid, as if some of the ichorous pus had escaped into it from the neck; about one and a half inches below the vocal chords there is an unmistakable pseudo-membrane; below this, near the bifurcation, the trachea has a bright-red color, as if a pseudo-membrane had been peeled from it, leaving the surface raw. The detachment of a pseudo-membrane from this part, if it did occur, must have been ante-mortem, for the organ had been carefully handled {514} in making the autopsy. Between the apex of the left lung and the median line the tissues of the neck, dissected upward, are found indurated, yellow, and giving an offensive odor, showing that the cervical cellulitis had extended downward farther than usual. The bronchial glands have undergone hyperplasia, being enlarged and hard. The right lung is normal; about one-half of the left lower lobe is consolidated, and when cut is found to be gangrenous and offensive. The liver is apparently somewhat enlarged; spleen normal in size; gastric mucous membrane has a congested appearance and is covered with mucus; mesenteric glands enlarged, pale, and firm; Peyer's patches swollen and pale; at lower end of ileum some pigmentation of these glands; in large intestine the solitary glands are enlarged, and a few of them pigmented; kidneys pale, cortex thickened, and markings indistinct. Microscopical Examination.--In the pia mater perhaps a little increase of cells; meninges of brain otherwise normal. The trachea shows well-marked diphtheritic inflammation; it contains a film of pseudo-membrane; evidences of inflammation occur also upon the laryngeal surface, though less marked than in the trachea. The solidified portion of the lung exhibits the ordinary lesions of broncho-pneumonia, with some interstitial change. In the kidneys we find parenchymatous nephritis, with some cell-growth in the Malpighian bodies.

The above case has been related at length, not only because it shows how severe and destructive the inflammation of the throat, extending into the tissues of the neck, sometimes is, but because four other complications or sequelæ were also present--to wit, otitis media, diphtheria, nephritis, and pneumonia. We see from the above case how formidable a disease scarlet fever sometimes is when attended by the inflammations to which it so frequently gives rise, for a child older and stronger than this, if thus affected, would necessarily have perished with the best possible treatment.

In localities where diphtheria is endemic, as in New York City and Paris, scarlet fever is often complicated by a pseudo-membranous inflammation of the fauces and air-passages. In severe cases of scarlet fever the Schneiderian as well as the faucial surface is covered with it, so that it can be readily seen on inspecting the anterior nares. Occasionally, the pseudo-membrane appears upon the laryngeal and tracheal surfaces, as in the case which I have related above and in others presently to be related, causing dangerous embarrassment of respiration. This complication sometimes begins almost at the commencement of scarlet fever, but in most instances it does not occur before the third or fourth day, and it sometimes does not appear till in the declining stage of the fever. When it begins, it intensifies the febrile movement and produces general aggravation of symptoms.

The common opinion is, that whenever a pseudo-membrane occurs upon the inflamed mucous surface in scarlatina true diphtheria has supervened; but there are those who hold that scarlet fever itself, when the inflammations which attend it are severe, may give rise to pseudo-membranes, so that what seems to be diphtheritic is but an element in the primary disease. My convictions are strong that when pseudo-membranes occur on any of the inflamed mucous surfaces in scarlet fever, true diphtheria has, with few exceptions, supervened if the patient live in a {515} locality where diphtheria is prevalent. That scarlet fever may occur in an individual along with another acute infectious malady is shown by abundant cases. It often occurs with varicella, and J. Herzog relates the following case, in which measles and scarlet fever coexisted: A boy aged eight years had measles, with the usual catarrhal symptoms, and on the fourth day, as the temperature was returning to the normal, it rose again suddenly, and the scarlatinal rash and sore throat appeared. In due time these subsided, and desquamation occurred. I have seen a similar case in consultation during the current year, so that there is nothing improbable in the theory that scarlet fever may coexist with other infectious maladies; and it is admitted that diphtheria, like erysipelas, may complicate the most diverse constitutional diseases. Moreover, when a child with pertussis, measles, typhoid fever, or tuberculosis suddenly develops a high fever with the occurrence of a pseudo-membranous inflammation upon the fauces or air-passages, all admit that diphtheria has supervened, since such inflammation is not an element in any form or type of either of these diseases; and I see no reason in the nature of the disease why scarlet fever should not be equally liable to this complication.

The elaborate treatise by Sanné of Paris on diphtheria contains a chapter entitled "Secondary Diphtheria." In it the author says, what all who are familiar with diphtheria will agree to, that secondary diphtheria does not differ in nature from the primary form, and that it exhibits a tendency "to occupy the organs which are themselves the seat of the more pronounced local determinations of the primitive malady.... Diphtheria is seen in the course or sequel of numerous diseases. Some appear to have a special proclivity for engendering diphtheria; these are specific maladies: measles, scarlet fever, pertussis." I have tabulated as follows Sanné's statistics of secondary diphtheria:

Cases Deaths Cures Doubtful ----- ------ ----- -------- Diphtheria complicating measles, 100 83 15 2 " " scarlet fever, 43 22 17 4 " " pertussis, 20 12 6 2 " " typhoid fever, 8 8 " " tuberculosis, 19 19

Sanné's statistics relating to the seat of scarlatinous diphtheria are as follows:

Fauces alone attacked, 15 cases. " with larynx " 4 " " " nasal fossa " 8 " " " larynx and nasal fossa " 4 " " " larynx and bronchi " 1 " " " nasal fossa and lips " 1 " " " lips and skin " 1 " " unaffected, 3 " Diphtheria generalized, 2 " Larynx only affected, 2 " Nasal fossa " 1 "

The opinion of so good an observer as Sanné, that when in scarlet fever, pseudo-membranous exudation appears upon the mucous surfaces which are the seat of scarlatinous inflammation, diphtheria has supervened, and not a croupous form of scarlatinous phlegmasia, carries with it great {516} weight. That it was diphtheria in four instances in my practice I had sufficient proof, for this disease became dissociated from scarlet fever, and extended to other members of these families as idiopathic diphtheria.

Nevertheless, one of the most difficult problems which we have to deal with in certain cases is to distinguish diphtheritic from non-diphtheritic inflammation; and I see no reason why the scarlatinous inflammation when intense may not be sometimes membranous; and those no doubt err who ignore this, and consider every inflammation attended by a pellicular exudation diphtheritic. We know that in some cases of dysentery a fibrinous exudation occurs upon the surface of the colon; that in croupous pneumonia fibrin exudes into the bronchioles and alveoli of the lungs; and that physicians in localities where there is no diphtheria meet, though at long intervals, cases which they designate croupous pharyngitis and laryngitis; and it seems to me that the intense inflammation of anginose scarlatina probably sometimes produces the same exudation. Moreover, it is very difficult to distinguish in the swollen fauces between a membranous exudation and ulceration or superficial gangrene so common in malignant scarlet fever. The grayish-white surface, jagged and foul, may be the one or the other, an exudation or a sphacelus, and in certain instances it is impossible to discriminate between the two conditions at the bedside.

Diphtheria complicating scarlet fever sometimes begins nearly simultaneously with the latter. Henoch states that exceptionally he has observed suspicious patches upon the fauces before the appearance of the scarlatinous eruption upon the skin; and he adds: "I have had repeated opportunities of observing this unusual beginning. In such cases we must ask ourselves whether the first affection was really connected with the second, or whether the former was a true primary diphtheria, rapidly followed by scarlatina. This opinion is favored by the fact that I have only observed such cases in the hospital, in which infection with various forms of contagion can scarcely be avoided."