PEMPHIGUS CHRONICUS VULGARIS OF THE LARYNX AND MOUTH. BY J. H. BRYAN, M.D., WASHINGTON, D. C. ■Mete YTortt l&efucal for November 25, 1899. BBPBINTBD FBOM THE Reprinted from the New York Medical Journal for November 25, 1899. PEMPHIGUS CHRONICUS VULGARIS OF THE LARYNX AND MOUTH * J. H. BUY AN, M. D., The following brief sketch of a case of pemphigus chronicus vulgaris affecting only the mucous membrane of the larynx and mouth is of interest on account of the rarity of this affection. In this country, at least, it must be extremely uncom- mon, for I am familiar with only one or two reports of cases by American authors, the most noticeable one being by Dr. Lewis H. Miller, of Brooklyn, who, in the New York Medical Journal for July 3, 1897, reports one case. The foreign literature is, however, much more re- plete, especially that of the Germans. The younger Schrotter, however, does not consider it such an un- common affection, having had a personal experience of five cases. WASHINGTON, D. C. Mrs. consulted me in October, 1898, for the first time, complaining of certain indefinite sensations in the region of the larynx, and stated that for six months or * Read before the American Laryngological Association at its twenty- first annual congress. Copyright, 1899, by D. Appleton and Company. 2 PEMPHIGUS OF THE LARYNX AND MOUTH. more she had had trouble with the throat. The attacks were frequent, but not of long duration, the principal sensation being that of a foreign substance in the larynx. She has always enjoyed good health, save for occa- sional attacks of muscular rheumatism, which have not been severe enough to occasion her much inconvenience. Her appearance gave me the impression of a well- nourished person, although a little paler than usual. The examination showed the nose, nasopharynx, and pharynx in good condition; but with the mirror a small white membranous deposit, about a quarter of an inch in diameter, was observed on the laryngeal surface of the right half of the epiglottis. While in the act of making a local application with a cotton-tipped applicator, this deposit was detached and brought away on the cotton. The membrane be- neath was red, but it did not show any loss of substance such as is met with in the various forms of ulceration that affect mucous surfaces. I sent this piece of mem- brane, which was of considerable thickness, to Dr. Jona- than Wright, requesting him to make an examination of it for me, at the same time venturing the opinion that we had in all probability a case of simple mem- branous laryngitis to deal with. I tried also to reas- sure the patient her affliction would soon be a thing of the past. In less than four days she returned to the office saying the trouble had returned, and she felt it on the left side of the throat, referring to the larynx. On examination a deposit of membrane of the same character, and about the same size, was observed on the left half of the laryngeal surface of the epiglottis—the seat of the former deposit looked perfectly normal. About this time I received a statement from Dr. Wright, giving it as his opinion the case was one of chronic pemphigus vulgaris, and also giving me the re- sults of his microscopical and bacteriological examina- tions, which were as follows: Under the microscope the membrane showed a fibrinous deposit containing numer- PEMPHIGUS OF THE LARYNX AND MOUTH. 3 ous round cells, but no epithelium; staining with Gram’s method showed a large number of cocci, but no bacilli. I am free to confess that up to this time I was not familiar with this condition, never having seen a case before. There have been frequent outbreaks since the original observation, the membranous deposit making its appearance on one half of the epiglottis, disappear- ing in a few days to reappear on the other half of this cartilage. The patient denies ever having had any cutaneous disease. About three weeks ago she complained of feel- ing much worse, especially of being very nervous and weak. The examination revealed a small deposit on the epiglottis, and for the first time the gums were noticed to be quite red and swollen. There was a membranous deposit on the upper left half of the gum about an inch in length, and a smaller deposit on the lower gum just below the incisor teeth. She was a little more anaemic looking, and she complained of a slight swelling of the lower extremities. An examination of the heart showed nothing abnor- mal, except that the sounds were not quite as clear as they should be in a vigorous person. A urinary analysis showed a slight trace of albumin with a few blood-corpuscles and pus cells, and a few granular hyaline casts. An examination of the blood showed nothing ab- normal. Pemphigus is a varied form of skin affection char- acterized by the formation of bulla;, and whether it be of the benign or malignant variety dermatologists con- sider it a very rare disease. The eruption on the mu- cous membrane of the upper air-passages is noticed in all forms of the disease, being more common in the chronic than in the acute variety. It is generally sec- ondary to the skin eruption, gradually extending into the mouth, pharynx, larynx, and into the trachea and 4 PEMPHIGUS OF THE LARYNX AND MOUTH. bronchi; and also occasionally affecting the conjunctiva. There are, however, a number of instances in which the eruption makes its appearance on the mucous surfaces primarily, as in my case; and cases are cited by Mes- nard, Bleibtreu, Penrose, Carre, and Miller. Generally the appearance of the eruption on the mucous mem- brane is characterized by the formation of a bleb, which is filled with a yellowish fluid such as we find in the bullous formations on the skin. This bleb finally rup- tures and a milky white membranous deposit remains. It is questionable whether the appearance of the erup- tion on the membrane is always accompanied by the formation of bullse". In my case I have never been able to recognize them, although the patient is able to deter- mine the time of the appearance of the eruption on the membrane by a pricking sensation in the larynx, and she has been in my office within half an hour of this time. According to Chiari, the bullas are the result of a rapid exudation, while in a slow exudation it simply causes a raising and discoloration of the epithelium, giving the grayish deposit the appearance of a diph- theritic membrane. Acute pemphigus of the mucous membrane is always accompanied by a high fever, while the chronic variety is generally without fever, and oc- curs in those who feel otherwise well. The diagnosis of pemphigus of the larynx, when it is secondary to the skin eruption, offers little or no diffi- culty, but it is quite another matter when it makes its appearance primarily on the mucous membrane. It is to be distinguished from diphtheria, tuberculosis, syphilis, herpes of the larynx, and finally the caustic effects of acids or lye. PEMPHIGUS OF THE LARYNX AND MOUTH. 5 The aetiology of pemphigus is extremely obscure, the majority of authors holding to the tropho-neurotic the- ory. Microscopic and bacteriological examinations have been made in Mandelstamm’s, in Miller’s, and in my case, all of which were of a negative character. This is essentially a chronic disease, and may last months before disappearing or wearing the patient out. Local applications have no influence whatsoever, alkaline washes probably giving the patient some relief from the constant irritation in the mouth and larynx. The only remedy which is supposed to have the slightest influence on the disease is arsenic, either in the form of the Asiatic pill or Fowler’s solution carried to the point of tolerance. This latter remedy has acted well in my case. The patient at the time of writing has not had an outbreak either in the larynx or mouth for two months. Physicians who desire to keep au courant with the advances of medicine and surgery should read The New York Medical Journal Subscription price, per annum, $5.00 Volumes begin in JANUARY and JULY D. APPLETON AND COMPANY, Publishers 72 Fifth Avenue, New York