Oral manifestations and treatment of pemphigus vegetans

Oral manifestations and treatment of pemphigus vegetans

Oral ORAL MANIFESTATIONS Medicine AND TREATMENT VEGETANS HARRY I,. LEVIN, COMMANDER (IX) OF PFJbIPHIGUS USNR” FTER an extensive review of the lit...

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Oral ORAL MANIFESTATIONS

Medicine AND TREATMENT VEGETANS

HARRY I,. LEVIN, COMMANDER (IX)

OF PFJbIPHIGUS

USNR”

FTER an extensive review of the literature, and from personal experience A with several cases of pemphigus in my own practice of dentistry, I had become interested in the several aspects of this interesting and often fatal skin disease with oral manifestations. Although the concept of pemphigus dates back to McBride,24 who described this disease in 1791, its value is impaired because the present concept of pemphigus is recent in origin. During the last century only two writers (Hebraz5 in 1860 and Brocq3 in 1902) reviewed the history of pemphigus in their respective textbooks. Their reviews were brief and, so far as the earlier history is concerned, the older sources were cited without regard to the change which had taken place in the concept of pemphigus. Due to the rarity of the vegetans variety of pemphigus, it was felt that a case which I now have under my observation was worthy of reporting. It is hoped, too, that the dental surgeon will be apprised of this rare and often fatal disease and all its ramifications, types, histopathology, treatment, and prognosis, since it may come under his scrutiny during a professional lifetime. Etiology In the true forms of pemphigus no specific organism has been found to be considered an etiological factor. Many theories have been propounded, but all have been found wanting. All have their supporters, but as yet no one theory is convincing or sustained by results. Pemphigus as a disease. comprises well-known types; the most common is pemphigus vulgaris and the less common types are pemphigus foliaceus and pemphigus vegetans. A variant disease of pemphigus foliaceus is pemphigus erythematosus, first described by Senear and Usher.l The vulgaris type, in its most virulent form, usually terminates in death from six months to one year after its onset unless it passes into either the foliaceus or the vegetans type, which is not so fatal. Pemphigus conjunctivae (benign mucous membrane) is not a true pemphigus because it is rarely fatal and is lacking the property of The opinions or assertions contained herein are not to be construed as official or reflecting the views Service at large. *United States Naval Receiving Station, Boston, 742

the private Ones of the of the Navy Department Massachusetts.

writer and are or the Naval

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acantholysis. It may undergo spontaneous and permanent remission, occasionally altering its course to assume the more virulent form of the true pemphigus with eventually fatal outcome.2 Finally, there is a sixth type which is not :J pemphigus in the strict sense of the word but, rather, a bullous pemphigoid. since it is without acantholysis and is not extremely serious, as it runs a mild chronic course. Pemphigus occurs in persons of all races and is about, equall! divided between men and women. Its more virulent forms are most commonl! seen in the Jewish people and particularly in persons of Southeasbern European origin. Furthermore, Brocq3 has suggested a further division of pemphigus vulgaris into two types-a subacute malignant type or butcher’s pemphigus (pemphigus vulgaris malin), which is no longer regarded as a disease entity, and a benign chronic type. The significant initial manifestations of pemphigus are characterized 1,) flaccid bullae that appear upon the normal mucous membrane, break easil)., and leave weeping, eroded, and hemorrhagic surfaces. The fluid in the bullac is clear or straw-colored and later may become turbid or purulent. The mov+ merit, of the epidermis over t.he dermis demon&rates the so-called Nikolsky sign (named for V. P. Nikolsky of Warsaw, who first described this strange phenomenon at the turn of the century). Much stress has been placed on acantholysis, the cleavage of the epidermis at t,he suprabasal level, and the liberation of the acantholyt.ic cells in the. bulla as being pathognomonic features in the sections of pemphigus. At the onset, due to t,he involvement of the oral mucous membrane, mast,ication becomes seriously impeded. With further extension of the intlammation into the larynx, hoarseness results. Conservative treatment by the dCntil1 surgeon with antibiotics and local palliativrs is ineffe.ctive. If the condition remains refraetorp and a favorable response is not forthcoming after scvpral days, the dental surgeon’s suspicion must be aroused and he must not lose an: time in referring the patient to the internist or dermatologist for further trca,tment and evaluation. Usually the oral manifestations are associated with bullar that appear upon other surfaces of the body, especially the armpits, groins, genitals in the male, and under the breast and sometimes upon the mucous membrane of the vagina of the female. Oral condit.ions might prrcndcx t’hcb latter manifestations by many months. Lever and Talbott4 state: “The essential histologic picture of this discast: is the loss of t,he intercellular bridges and other degenerated changes in t.he c~;lls of the lower epidermis, leading to acantholysis and formation of clefts and bullae within the lower epidermis. The bullae are located predominantly in the suprabasal position with only the basal layer remaining adherent to t,hckvorium in pemphigus vulgaris type. This is because t,he coherence between the hasal layer and the corium is brought about not by the intercellular bridges but, ot.htbr elements, namely by cytoplasmie processes of the basal cells fitting into spacers of reticulum fibres. ” Civatte5 points out the characteristic histologic features of the buliac: of pemphigus in which the epithelial cells of the basal layer lose their intercellular bridges. The cells themselves are not greatly interfered with, since t,he nnclri of the individual cells do not seem to be impaired and the cytoplasm at the

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periphery is slightly thickened. The intercellular layers of the epidermis account for the bullae forming from the fluid collection during the loss of the cellular cohesion. Civatte called this destruction of the intercellular bridges acantholy&, which is typical of pemphigus. Tzanck+” went on to propose a simple procedure which supplements the examination of the histologic sections of the bullae. By scraping the floor of the In this way bullae, one may obtain cellular material for cytologic examination. Tzanck’s test demonstrates the profusion of epithelial cells in the bullae of true pemphigus. The acantholytic epithelial cells have a rounded shape; their peripheral condensation of cytoplasm is in direct proportion to the nucleus. In contrast to smears from pemphigus bullae, the smears from the base of a bulla of dermatitis herpetiformis show a predominence of inflammatory cells and all other cells are relatively scarce. According to Blank,llg 26 tonofibrils in the living material can be seen with a phase microscope, as first suggested by Dr. William Montagne, Department of Biology, Brown University. In addition, they can be seen under certain conditions in the electron microscope. However, there is still the controversy as to whether the epidermal cells of the syncytium, or whether the fibrils are merely fibers connecting the cells. Whatever the structures, they are missing, defective, or damaged in some way in the epidermis of the patient with pemphigus. This, of course, accounts for the epidermolysis which is so characteristic The cause of the loss of the loss of these fibers is unknown of true pemphigus. but, nonetheless, this loss is clearly visible under the searching lens of the microscope. Even though acantholysis also occurs in some other diseases, such as herpes simplex and herpes zoster, one should not infer that there is any relationship of pemphigus to a virus infection. Perhaps at this time a review of the discerning differences in the various types of pemphigus is in order.

Pemphigus Vulgaris.-Acute pemphigus vulgaris (Figs. 1 and 2)) has bullae which are usually small and flaccid; these break easily, leaving erosions which tend t’o enlarge as the epithelium becomes detached at the periphery. By peripheral extensions and confluences of the erosions, large areas of the body become denuded, which is so characteristic of this type of pemphigus and the lack of tendency for t,he erosions t,o heal. The oral mucosa is involved in all cases. The mouth is frequently the site of the first lesions. The lesions are usually severe and peripheral and eventually may involve the entire mucous The lesions from the oral cavity membrane of the mout,h, pharynx, and larynx do not stop at the vermilion border of t,he lips, but continue to form excoriations on the chin, neck, and front of the body; in fad, they may cover the entire body. Histologica.lly, it can be seen that hyperkeratosis is a prominent feature with a mild inflammatory condition oi t.lre submucosa. There are occasions when the submucosa may be rclativcly free of infiammat,ory cells. ICist,ologic section of the involved epithelium reveals that the. separation of the epithelium is just above the basal-cell layer. The bullae, it will be seen, are filled with acanthoid epithelial cells and an occasional inflammatory cell.

Fig.

1.

E’ig. l.-Pemphigus vulgarisnig. Xnfl;mtnatc;ry cells in rete uegs of the tpitlltrf lrkeratosis is prominent and mild chronic inflammation is seen in the suhmuccsa. Fig. 2.--Pemghigus vulgarismg. Separation OP the epithelium above the baua. layer is revealed, Microabscess formations can be seen. Hyperkcratosis is a chnra.rtel featu re.

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Fig. 3.

Fig. 4. Fig. 3.-Pemphigus vegetans-62 mg. The marked features of this section are the ac lthosis and papillomatosis of the epithelium. (Courtesy of the Armed Forces Insti tute of ‘athology. 1 Fig. 4.-Pemphigus vegetans--62 mg. Note micro-abscess formations filled 1with eo sinophils in the dermis. (Courtesy of the Armed Forces Institute of Pathology.)

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Pemphigus Vegetans.-Pemphigus vegetans resembles pemphigus vulgaris in every respect, except for the vegetations which form secondarily on t,he esThe condition is grave, but not so hope.less as that of the acute coriations. condit,ion. Fruhwald,” in review of 147 eases of pemphigus vegetans as reported in the literature, found the mortalit,y rate to be 78 per cent. Oral lesions arc formed in almost every case and the disease usually starts in the oral cavit?., Vegctatioris 011 where it may be the initial lesion of this type of pemphigus. the mucous membrane of the oral cavity are rare. If t,hey arc absent, the oral If they arc present, lesions are identical with those seen in pemphigus vulgaris. 1Jsually the vepet,ations art’ the veget,ations appear as soft, dark granulations. limit,ed to the skin. There is a diffuse, dense, chronic inflammatory extltlatt‘ which appears to he confined to the papillary area.

Fig. 5.-Pemphigus vegetans-95 fllled with eosinophil cells. Inflammatory Armed Forces Institute of Pathology,)

mg.

Micro-abscess cells are thick

may be seen in the basal-cell in the submucosa. (Courtesy

layer, of the

Histologically, the marked changes are the acanthosis and papillomatous epithelium with the formation of micro-abscesses of Munroe and, on occasion, spongiotic abscesses. In the vegetans type of pemphigus, the epithelium is separated above the basal-cell layer (Figs. 3, 4, and 5 ).

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Pemphigus Foliaceus.-Pemphigus

foliaceus (Figs. 6 and 7) shows bullae on t,he skin in the early stages and usually assumes the appearance of a generalized exfoliative dermatitis, from which it is easily dift’crcntiatcd, however, by the Nikolsky sign. The upper layers of t,he epidermis can bc rubbed off by a stroke of the thumb. The mortality rate is 56 per cent. TAever’” informs us that the mortality rate in his thirty eases was 60 per cent. Oral lesions in pemphigus foliaceus are uncommon. In fact, many authorities believe that oral lesions may not occur at all. In pemphigus foliaceus there is an exfoliation in the early stages without the development of denudation of the epithelium, and in the latter stages a generalized scaling and crusting superficially occurs. The scales are small and flaky. The underlying areas of the healed lesions present, pigmented patches of a light brown color. The histologic picture presented from sections of the pemphigus foliaceus Hyperkeratosis bullae shows acanthosis in the upper portion of the. epidermis. is again a prominent feature. Detached epithelial cells may be seen here and there in the bullae, when present, and even in the clefts.

Pemphigus Erythematoides.-Gray14

noted histologically that pemphigus erythematoides was very much like the foliaceus variety when this form of pemphigus became localized. It was also noted that this form of pemphigus might even change to the virulent types of pemphigus foliaceus or even pcmphigus vulgaris. The mortality rate is negligible, but when the disease passes into the more virulent form the picture changes. No oral lesions have been observed.

Pemphigus Conjunctivae (Benign Mucous Membrane).-The reasons for the separate consideration of this form of pemphigus arc the predilection of the lesion for mucous membrane, the tendency to produrc scarring of’ the eyes and other mucous membranes, and the benign chronic course of the disease. Pemphigus conjunctivae always attacks the mucous membrane, and in about one-half of the cases it attacks the skin. The conjunctivae are nearly always affected and The lesions always produce occasionally present the only area of involvement. scarring of the conjunctivae, frequently scarring of the other mucous membranes, and occasionally scarring of the skin. Oral lesions were present, in Blisters, which do not break easily, twenty-seven of Lever’+” thirty patients. are scattered over the mucosa of the oral cavity. When the blisters do manage to break, the ensuing erosions do not enlarge; the.y do not tend to heal well, but persist for months. The lesions are only moderately tender and appear cleaner. They do not cause any peripheral expansion, nor do they bleed or ooze easily. Histologically, this type of pemphigus does not produce acantholysis. Hence, it may not be considered a true pemphigus, but is known rather as a pemphigoid benign mucous membrane. Another type of mucous membrane pemphigoid is the Senear-lisher syndrome16 which usually appears on the face as a butterfly design, the body of which straddles the nose. The primary lesions are the bullae, which are usually small and flaccid, and their thin-roofed wall which tends to rupture easily, giving rise to two other types. The first of these is the reddish raw patch of epidermis.

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l3ullae are larger and are located in tht! ui Fig. 6.-Pemphigus foliaceus--65 mg. of the prickle-cell layer. (collrtcs) Hyperkeratosis is a prominent characteristic. PO1tion the Armed Forces Institute of Pathology.) Fig. 7.-Pemphigus foliaceus-85 mg. Hyperkeratosis and bullae are noted. ‘l%rrc ma ny inflammatory cells in the submucosa. Hcrc ant1 thrrc are small abcesses. Of the Armed Forces Institute of Pathology.)

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As a result of coalescence of these lesions, the second variety consists of lesions covered with thin, greasy, yellowish, scaling crusts. These involute, as a rule, leaving pigmented patches.

Treatment Since the introduction ol corticotropin (ACTH) and cortisone in 1949 by Hench and his co-workers”Y at the Mayo Clinic, their scope of application has spread rapidly with amazing and gratifying results. Among the diseases of interest to the dermatologist, disseminate lupus erythematosus and psioriasis were the first in which the effects of these hormones were reported. Since then, numerous other dermatologic conditions have been treated and, although experience is still limited, it is expanding daily. The advent of ACTH and cortisone has completely revolutionized the treatment of all types of pemphigus and has decidedly improved t>he prognosis of the malignant type which hitherto baffled all treatment and always ended fatally. Both ACTH and cortisone are effective, hut ACTH is found to be seven times It is advisable, therefore, to more potent intravenously than int,ramuscularly. give ACTH intravenously in a 5 per cent glucose saline solution in the very severe cases of pemphigus. However, for the ambulatory patient and for cases of remission such therapy might not, be expedient or practicable. In such cases Cortone is very effective, even when the patient can be treated by prescription. given orally in low minimal doses sufficient to maintain the remission. 0 ‘Leary18 noted that the improvement that follows the use of ACTH and cortisone in most diseases of the skin is of short duration. Maintenance t,herapy may control, but does not cure, any of the c,hronic dermatoses of a serious nature. In order to produce even temporarily successful effects, the physiologic actions of the drug must be understood. 0 ‘Leary further intimat,es that the. treatment of pemphigus by ACTH and cortisone does not give constant results. A few patients will derive considerable relief because new bullae will cease to form after the administration of ACTH and cortisone in sufficiently high therapeutic doses; some patients will experience a remission and remain free of new lesions as long as they are kept on maintenance doses; and others do not obtain relief from the disease, and death will follow an uninterrupted course. It would appear that in cases of pemphigus foliaceus the remissions last longer and are more pronounced than in cases of pemphigus vulgaris. For the ulcerated skin surfaces, hydrocortone ointment in 1 to 5 per cent dosages has been used. When hydrocortone ointment was used alone, the results are disappointing and ineffectual; hence, the ointment must be used only as an adjunct therapeutic agent together with the internal medication. In extremely toxic states whole blood infusions will sometimes give relief. ACTH in form of a gel will give a protracted and accumulative effect which results in the uninterrupted stimulation of the adrenal glands. The lifesaving qualities of ACTH and cortisone make it the choice therapeutic treatment. While it may not actually save life in the full meaning of the word, it does prolong life and all authorities are agreed that remissions are

brought about and maintained for long periods of time. Remissions last a*‘? where from several months to many years, depending upon the type of ptXmphi gus. Patients who at one time were moribund are now useful, economicall! A 1011~ self-satisfying persons through the agency of ACTH and cortisone. with the treatment, secondary infections can be controlled by the applicat iof of antibiotics; the patient can pursue a normal way of life and enjoy cornpar: tively good health if treatment prophylactically by one or t,wo doses of’ effect.ivc minimal doses of ACTH and cortisone is given daily. (lombes and associateslg makes special ment,ion of the fact t,hat p,ctmphigu: vegetans was the type of pemphigus most responsive to ACTW. While it was true that thee new drugs wer(h used empirically at the> bt> ginning, a satisfying regimen has been worked out in the treatment necessar: to bring about an abatement of tht> virulent condition to one of remission. 7% antibiotics definitely controlled the secondary infrrtions, but, were of lit1 le 01 no value in the t,reatment of the bullous states, although several dermatologist: have stated that remissions have been obtained with the use of Aureoniycdit1 MaaddawizO regarded the treatment of pemphigus ineffective when hc ernploycSt this antibiotic in several cases of pemphigus vegetans. (2~tigestive heart. failure and hypertensive cardiovasc&r disease ulay Ire In part, this appears to be due 1.0 salt alto aggravated by ACTH treatment. water retention and peripheral resist,anrc secondary t.o t.hr administration OCt h( ACTH preparation. Cort,isone has betxn ?;hown to inc~recse blood pressnrr tlvtlt in hypertensive patients. In patients with hirsutism and acne, these tlisturbanccs are likely to Ire aggravated by prolonged ACTH and cortisone tl~erapy. In ost,eoporosis ilni osteomalacia, it is assumed that the negative nitrogen balance created by A(‘TII and cortisone therapy and the calcium and phosphorus loss may aggrnvatc t ht condition unless specific factors assoriatal with osteoma.la& and ostq)orosi! are corrected during therapy. The most common reactions one must, be on guard against, arc: wt.c~xith of fluid leading to cardiac decompensation (best averted by I he rest ric*tion OI sodium chloride intake), hypokaliemic alkalosis (prevented by prophylwtic administration of potassium chloride, 2 to G (in). per day by mouth), wac.tiw. tion of an old pulmonary tuberculosis or peptic ulcer, psychoses, irked WV(~IY In order to prevent severe, ovenvhclming infection in ill and dts. infection. bilitating patients during periods of treatment. wit.11 st.croids, it is atlvisahlcl 1~ administer concomitantly an antibiotic drug, &her penicillin, 300.000 unit-~. per cubic centimeter, Aureomyein, 250 trig. per day, chloromyretin, or Terramycin 2 Gm. per day. In patients receiving corticotropin (A(fTH) irrt rav~~nously, it is most convenient to add 300,000 unit,s per cubic cent inwt.tlt* of Iwrticillin directly to the infusion mixture. Kinsell and associates*’ say t.liat the ideal arrangement, in Ihe aduliniHt ration of the hormones, ACTH and cortisone, is to give heavy dosages at, the bcginning to control the ravages of the disease and, when remission is attained, then taper the minimal doses to almost. the point where the hormone can hc tliswmt,inued altogether.

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Since the action of this drug is only suppressive, it is expected that the withdrawal of this drug would cause a recurrence. of the original condition. Hence, to be practical, it should be kept up indefinitely. A very gradual withdrawal of the hormone is necessary if, for any reason, it must be discontinued, since abrupt withdrawal may lead to severe malaise, with depression, fatigability, anorexia, and weakness combined with the symptoms of relapse, and give rise to further disturbing illnesses.22 In suspected pemphigus it is important, to e.xcise an intact bulla. The early blister is more instructive and informative than the older one. One or two hours after their formation is t,hc ideal time for study, because secondary changes such as regeneration, degeneration, and secondary infections may obscure essential features of the disease. The history of the case and presumptive diagnosis, as well as t,he site of the lesions, are important in deriving at the proper diagnosis. The arguments for and against the use of t,he hormones for use in dermatologic conditions are voluminous. Conclusions are that they do not cure; nor have these hormones saved any lives in many fulminating diseases, such as lupus erythematosus, We can say that cortisone and ACTH have been beneficial in many diseases of a less serious nature. Supportive treatment, for pemphigus must depend on good nursing care and high calorie and high protein intake. If the patient should develop anemia, as many patients with pcmphigus do, whole blood infusions will sometimes help. The emotional reaction to t,hc use of’ corticotropin (ACTH) and cortisone is both good and bad. initially the patient is greatly encouraged when pain and pruritus abate. This also enhances the feeling of well-being when treated with these drugs, but when the administration is stopped, rheumatic pains recur sometimes with serious reactions of somewhat greater intensity than before treatment was undertaken. This leads to an emotional letdown; the patient becomes depressed, discouraged, and despondent. There are incidents of suicide attempts. To sum up tho purpose of this article, Schrieber’” relates a case in point, that of a ‘70-year-old white man. For five months the condition of his oral cavity was such that it failed to respond to conservative dental therapy for what was purported to be an ulcerated lesion of t)he mucosa. Not, until a biopsy was taken was the case diagnosed as pemphigus vegetans. The dental surgeon must realize that pathologic conditions of the oral cavity which fail to respond to either antibiotics, vitamins, and thorough prophylaxis a,fter a week or two should immediately become suspicious that the condition is general rather than localized. The patient must be transferred to his family physician, internist, or dermatologist who would be in a better position to deal with his condition.

Case Report M., a 33-year-old man, has been carried on continuous leave of absence for the past one and one-half years. He was admitted on June 23, 1954, for evaluation. Two years before admission, the patient’s skin broke out with pustules about the A short time later, vesicles appeared on the tongue and mucous memface and neck. branes of the mouth. At about the same time an erythematous eruption appeared in the J.

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groins and about the scrotum, which went on to vesicle formation and finally pustulation. The patient was incapacitated because of this difficulty and was hospitalized at 11:~s sachusetts General Hospital for one week and treated with local measures. Shortly thereafter he was admitted to the Boston Veterans Administration Hospitai for the first time, where, after biopsies, a diagnosis of pemphigus vegetans was math. The patient then was started on ACTH and later cortisone; he has remained on theat, although at no time has his skin bee I drugs ever since. He has had marked improvement, completely clear of these lesions (Figs. 8, 9, and 10). Past History.-The patient has had colitis, with frequent attacks of diarrhea in childhood, and his usual stable state is four or live bowel movements a day, with occasional blood. Six months before admission he had a flare-up with grossly bloody diarrhe:r. Bronchoscopy showed an inflamed mucosa without obvious ulceration, and bariuln c:nerr:;r was typical of extensive ulcerative colitis involving most of the colon.

Fig. 8.-Patient J. M., showing an inflammatory ridge of furunculosis at the collies, lint. of the neck. even though the patient is now in remission from pemphigus vegctans. Nc,tt. pearl-like bulla at the left center.

Fig. 9.--J.

M., a white

male patient

with

excoriations

of the mucous membrane

of the lins.

Physical Examination.-Temperature was 99.5” I?.; pulse, 100; blood pressure, -141),/90. The patient, an obese white man, was in no acute distress. The skin tended to be oily in general. There was a patchy erythema over the sternum and over the upper back. ‘I’here were small crops of furuncles and vesicles on erythematous bases around the mouth :~nd along the collar line, extending to the back of the neck. Just inside the hairbne of the

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right frontal area there were confluent areas of oozing vesicles with foul odor. In the left nasal fold there was a crusted area. The intertriginous areas of the groins and genitals were pigmented with a few vesicles. There was no lymphadenopathy. Examination of the head and neck revealed nothing remarkable, save for the mouth. The tongue was well papillated. The buccal mucosa was pale, granular, and deeply furred; over the soft palate were numerous small vesicles with a tendency to coalesce. The lungs were clear. The heart was normal. The abdomen was felt at the costal margin and was nontender. The remainder of physical examination was within normal limits.

Fig. 10.--J. M., a 33-year-old white man. The patient, in a state of remission from pemphigus, is not altogether free of ulrwations rm the nose at the alae anci on the side.

Labonat.0~ Data.-Hemoglobin was 10.8; hematocrit, X? per cent; white count, 8,000 Urinalysis was normal. Stools with 2 bands, 53 polys, 44 lymphocytes, 1 eosinophil. showed 2 to 3 plus occult blood, negative for ova and parasites. Negative phosphorus nitrogen was 34 mg. per cent. Serum proteius, 6.6; albumin, 3.0; globulin, 3.6, Liver chemistries showed serum bilirubin, 0.4 mg.; alkaline phosphatase, 5.3 Bodansky units. Cephalin flocculation was negative. Thymol turbidity was 8.3. Prothrombin time was 40 per cent. Serum electrolytes were within normal limits. Stool cultures were negative for pathogens. Chest x-ray was normal. There was no evidence of bone atrophy. Hospital Course.-The patient was maintained on ACTH and cortisone therapy; the only change in therapy was the addition of daily washes with pHisoHex and initiation of ultraviolet light to the skin of the face. The patient did well on this program and was discharged to leave of absence on July 10, 1954. While on leave of absence, the patient continued to do reasonably well; his therapy was changed in that cortisone was raised to 100 mg. per day and ACTHAR gel was lowered to 16 units per day. He was discharged from leave of absence on Aug. 11, 1954. (2) IJlcerative colitis; Diagnosis.-(1) Pemphigus vegetans; treat,ed, improved. treated, unchanged.

Discussion A case of pemphigus vegetans has been presented. The patient in this case has had several remissions, but the present one is of fifteen months’ duration.

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The skin of the face is still not altogether free of lesions. The lesions found ill the oral ca.vity developed from erupted vesicles and bullae which burst freel-. leaving bleeding and oozing excoriations covering the entire mucous membrancl before remission occurred. also seen were shreds of loosely hanging epithclial These lesions are frequently mistaken for Vincent’s stomatitis or tissue. The differclntia,l diagnosis can be made b; noting nlceromembra.nous gingivitis. that Vincent’s stomatitis has a predilection for the interdental papillac which are ultimately destroyed if untreated, leaving a grayish slough, interdental pain. salivation, fetid breath, and at t,imc+ adenopathy with a high fever. There is The ,S’+onever a.n,v vesicular or bullous formation in Vincent’s stomatitis. c&e& vincenti and fusiform bacilli are found as the causative agents, whilcl itr petnphigus t,hc etiology is unknown. In any chronic vesicular erosive stoma1 itis which remains refractory to trcatmcnt, prmphigus must, remain a possibility until proved otherwise,

iIs dentistry increases it,s scope in tht: fields of the healing arts, it must. assume greater responsibilities. Tt has hren proved in some dermatologic states, in such diseases as pemphigus, that dentistry can help materially in the dctermination of the proper diagnosis. While pemphigus is primarily a dermatosiss. it is of considerable interest, to t,ho dental clinician becausr oral lesions urn extremely prominent. Moreover, t hc: manifestations in the mouth generally precede the cutaneous picture by long periods of time. Acut,e pemphigus is distinguished clini~~ally by cxarly development of lesions of the mouth, a rapid fulminating course, and a high racial susceptibility (somcThere may be some changes in arid-base tirnes as high as 80 per cent Jewish). balance of the blood. Chronic pemphigus, on the other hand, progresses slowl~~ and has prolonged asymptomatic periods. Even though corticotropin (ACTH) and cortisone may not, fulfill all the therapeutic expectations to which recent enthusiasm has given rise, it, is evident t,hat, a new era in modern medicine has been opened. The fact remains that these hormones arc the most effective and useful drugs in comba,tting pemphigus a,n(‘J other allied condit,ions at our command toda.y. I wish to express my gratitude to Colonel Joseph I,. Vernier (DC), USA, Director of the Institute of Pathology, Washington, D.C., for the fine microphotographs of the various cases of pemphigus and for his consistent help in my previous efforts; to H. G. Campbell, Chief Registrar of the South Boston Veterans Administration Hospital, for relinquishing a detailed case history on my patient, J. M.; to the Massachusetts General Hospital for implementing the case history and for the many fine microsropic specimens of the varioup types of pemphigus; and to Professor Walter F. Lever for his patience and constructive I also wish to thank Lyon P. Strean, D.D.S., PhD., of Merck & Company, Inc., criticisms. Rahway, New Jersey, for his helpful suggestions and criticisms, and Miss E. i\. l)enuis. Medical Librarian at the United States Naval H0spita.l. Chelspa, Massachuset,ts, for m:lking my contribution possible.

References 1. Renear, F. E., and Usher, Lupus Erythematosus,

B.: An Unusual Type of Pemphigus Combining Arch. Dermat. & Syph. 13: 716, 1926.

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2. Frazier, C. N., Lever, W. F., and Keuper, C. S.: Response of a Patient With Malignant and Benign Pemphigus to Adrenocorticotropic Hormone and Cortisone, Am. J. M. SC. 222: 312, 1951. 3. Brocq, L.: Les Pemphigus, in Practique Dermatologic, Paris, 1902, Maison & Cie, p. 721. 4. Lever, W. F., and Talbott, J. H.: Pemphigus, A Historical Study, Arch. Dermat. & Syph. 46: 800, 1942. Diagnostic histopathologique A. : de la dermatite polymorphe douloureuse 5. Civatte, ou Maladie du Durhing-Brocq, Ann. dermat. et syph. 3:l cited by Lever, W. F.: Pemphigus; histopathologic study, A. M. A. Arch. Dermat. & Syph. 64: 727-753, 1951. immediate en dermatologie, Bull. Sot. franq. dermat. 6. Tzanck, A. : Le cytodiagnostic et syph. 2: 68, 1947. : Contribution a L’etude du cytodiagnostic im7. Tzanck, A., and Bourgeois-Garvadin mediate en dermatologie, Bull. Sot. frana. dermat. et syph. 1: 193, 1947. 8. Tzanck, A., Aron et Rosenczweig : Cytodiagnostic rapide modification de la methode de Pappenheim, Bull. Sot. frang. dermat. et syph. 12: 447, 1947. immediate en dermatologie, Ann. dermat. et syph. 8: 9. Tzanck, A. : Le cytodiagnostic 205, 1948. R. : Le “eyto diagnostic immediat” des dermatoses A., Aron-Brunetiere, 10. Tzanek, bulleuses, Gaz. med. port. 2: 667, 194Q. 11. Blank, H.: Personnel Correspondence. R.: Pemphigus Vegetans, Leipzig, 1915, Leopold Voss. 12. Fruhwald, 13. Lever, W. F.: Pemphigus, Medicine 31: 1, 1953. Type, Proc. Roy. Sot. Med. 31: 871, 1938. Gray, A. M. H.: Pemphigus of Senear-Usher Histopathologic Study, A. M. A. Arch. Dermat. 6 Syph. 2: Lever, W. F.: Pemphigus-A 46: 201, 1942. Arch. Dermat. & Syph. 66: 429-439, 1952. 16. Senear, F. E.: Chronic Pemphigus Vulgaris, 1952. E. C., Slocumb, C. H., and Polley, H. P.: The Effect of a 17. Hench, P. S., Kendall, Hormone of the Adrenal Cortex (17-hydroxy-ll-Dihydrocortieosterone; Compound E) and of Pituitary Adrenocorticotropic Hormone on Rheumatoid Arthritis ; Preliminary Report of Treatment, Proc. Staff Meet., Mayo Clin. 24: 187-197, 1949. Cortisone and Corticotropin (ACTH) in the Treatment of Cutaneous P.: 18. O’Leary, Diseases, Mayo Clinic, Rochester, Minn. Postgrad. Med. 12: 10-14, 1952. J. J., Simuango, S. S., and Canizaries, C.: Pemphigus Vulgaris; 19. Combes, F. C., Kaufman, A Clinico-Pathological Study of 100 Cases, Arch. Dermat. & Syph. 6‘2: 786, 1950. Observations on the Treatment of Pemphigus, J. Egypt. M. A. 37: H. El: 20. Maaddawi, 55-59, 1954. L. W., Partridge, J. W., Boling, L., and Forman, N.: Corticotropin (ACTH) 21. Kinsell, and Cortisone-Newer Concepts of Their Use, California Med. 78: 487-490, 1953. Cortisone and Hydrocortisone, Brit. M. J. 1: 588-589, 1952. 22. Editorial: Report of a Case of Pemphigus Vegetans, ORAL, SURG., ORAL MED., 8; 23. Schrieber, H. B.: ORAL PATH. 8: 611, 1955. Introduction to the Theory in Practice of the Art of Medicine, 24. McBride, D. : Methodical Addition to Dublin, Watson publ. 1777, vol. 1, p. 239; vol. 2, p- 493. On Diseases of Skin, translated and edited by C. H. Fogge and P. H. Pye25. Hebra, F.: Smith, London, 1868, New Syndenham Society, vol. 2, p. 361. Abnormal Cytology of Epithelial Cells in Pemphigus 26. Blank, H., and Burgoon, C. F.: Vulgaris: Diagnostic Aid, J. Invest. Dermat. 18: 213-223, 1952.