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Observation on pemphigus vegetans
Observation on pemphigus vegetans William BROOKE
C. Hurt, GENERAL
Lieutenant HOSPITAL,
Colonel, FORT
SAM
USA HOUSTON,
TEXAS
P
emphigus vegetans is rare in this country. Therefore, it is difficult to gather material for a study of this disease. The purpose of the present article is to compare the histopathologic and clinical findings in pemphigus vegetans with those in pemphigus vulgaris, using material from the files at Brooke General Hospital and Womack Army Hospital. Pemphigus vegetans is of particular interest to dentists, since it begins in the oral cavity in more than 50 per cent of the cases. Often the oral lesions persist for months before skin lesions appear. It is during this time that the patient may consult his dentist for diagnosis and treatment. The need for early diagnosis in pemphigus has been stressed by Lever and White.l If the diagnosis is made soon after the onset, early trea.tment with corticosteroids materially improves the prognosis. According to Lever,2 there are four basic variants of pemphigus: (1) pemphigus vulgaris, (2) pemphigus vegetans, (3) pemphigus foliaceous, and (4) pemphigus erythematosus ( Senear-Usher syndrome). Of these four variants, pemphigus vegetans appears to be the most rare. Costello and associates3 reported fifty-two cases of pemphigus. Only one of the patients had pemphigus vegetans, whereas there were forty-two cases of pemphigus vulgaris, five cases of pemphigus foliaceous, and four cases of pemphigus erythematosus. More recently, Lever and White1 reported forty-six cases, only one of which was pemphigus vegetans. The histopathologic characteristics of skin lesions often pose diagnostic problems, but in pemphigus vegetans they are fairly distinctive. Perhaps Director4 has given the best interpretation of the microscopic changes. He believes that pemphigus vegetans is a reactive phase of pemphigus vulgaris which lends the patient increased resistance to the disease. In pemphigus vulgaris acantholysis, or loss of cohesion between the prickle cells, is seen. This results in the formation of cleavage vesicles within the not
The opinions or assertations to be construed as reflecting
contained the views
herein are the private ones of the author of the Department of the Army.
and
are
481
482
O.P., O.M. & 0.1’.
Ilutf
Octolwr,
1965
+dermis, just above the basal-cell layer. ‘I’hr~se vesicles Ina?- contain neutrophils and, in some instancrs, eosinophils. Also seen in these vesicles, on occasion, a?e the so-called Tzanck cells, which arc loosenetl prickle cells that, have floated free and are in various stages of degeneration (Fig. 1). The vesicles expand, forming large bullae. The rupture of these bullae results in loss of fluid, salts, and protein since, after rupture, the remaining surface cells break down, leaving large ulcerations (Fig. 2). Since the bullae cover large areas of the body surface, t,he loss of these elements and the likelihood of secondary bacttlrial invasion become serious problems.
Fig.
1. Photomicrograph
Fig.
Z. Pemphigus
of bulla
vulgaris
with
formation
extensive
in pemphigus
skin
involvement.
vulgaris.
Volume Number
20 4
Pemphigus
vegetans
483
In pemphigus vegetans the basic histopathologic changes are the same as in pemphigus vulgaris, but there are several important differences. Unlike pemphigus vulgaris, pemphigus vegetans is characterized by hyperplasia of the epidermis, especially the rete pegs or ridges. This hyperplasia, in part, prevents the breakdown of the surface epithelium. Acantholysis and vesicle formation are still seen, just as in pemphigus vulgaris (Fig. 4), but the thicker epithelium prevents spreading of the vesicle and large bulla formaCon. Fig. 3 is a composite drawing of the changes that may occur in the skin. The vesicle formed at C may be smothered by the regrowth of the hyperplastic cpithelium and carried toward the surface. Therefore, vesicles may be seen anywhere between the suprabasilar layer and the keratinized layer. As the vesicles migrate through the epithelinm, they may collect eosinophils that have wandered from the subepithelial connective tissue through the loosened epidermal cells. This results in the focal areas of eosinophils and neutrophils that are called microabscesscs(Figs. 3,B and 5). While there may be small areas of ulceration in pemphigus vegetans, the hyperplasia prevents massive exposure of underlying connective tissue and therefore keeps fluid, protein, and salts from escaping. Secondary bacterial invasion is also minimized. In the skin, the hyperplasia may throw up folds or irregular ridges (Fig. 6). Between the folds are troughs containing keratin and the contents of shedding microabscesses(Figs. 34, 6, and ‘7). These ridges are seen clinically as vegetations (Fig. 8). In the oral mucosa the hyperplasia of the rctc pegs is marked and may form a retiform pattern (Fig. 9). While actual folds are not seen in the oral mucous membrane, the surface may be rough and irregular. Acantholysis is demonstrable, and eosinophilic migration may be striking. Microabscesscscontaining eosinophils
Fig. 3. Composite drawing of histopathologic changes seen in skin affected vegetans. At C a vesicle has formed. At B a vesicle has been carried toward hyperplastic epithelium; it has collected many eosinophils and may be termed At B a microabscess is being shed.
by pemphigus the surface by a microabscess.
and perhaps a few neutrophils are part of the picture (Fig. IO). Eosinophils may be seen within the blood vessels and connective tissue of the submucosa (Fig. 11). Most authorities a.gree tha.t there is very little change: except for edema in the connective tissue underlying the affected epithelinm, in either pcmphigus vegetans or vulgaris. The histologic changes in the skin arc manifested clinically as dark brown or reddish fungoid masses (Fi,.w 8). These may u-ecp Prom the fissures and folds and then crust over. Although these vegetations do not cover t.he entire body surface, they may cover large arcas of it. The clinical lesions of the oral rnncosa often precede the skin lesions by months. The gingiva, buccal mucosa, palate, and pharynx may be involved. The oral lesions form a scrpiginous pattern throughout the mouth (Fig. 12j. The surface of these lesions resembles pus and can be wiped off with a cotton swab, leaving a bright red, weeping base that, usually does not bleed and is not
Pig.
Fig. Pig.
4.
4. Vesicle formation in skin section 5. Microabscess containing eosinophils
of pemphigus vegetans. dthin the epidermis
in pemphig,rus
rcgctans.
Volume Number
Pemphigus
20 4
cegetans
485
P
Pig.
Pig. Fig.
6. Skin seqtion of pemphigus vegetans. 7. High-power view of shedding microabscess
Fig.
8. Clinical
photograph
of vegetations
in skin
section
seen in pemphigus
of pemphigus
vegetans.
vegetans.
486
O.K. O.,\l. h 0.1’. ol~tnh’r, lRfj5
Hurt
SUMMARY
The histopathologic and clinical findings in pemphigus vegetans are compared to he a variant with those in pemphigus vulgaris. Pemphigus regeta.ns appears
Fig.
Fig. Pig.
I)iopsy
10.
9. Gingivnl biopsy specimen of pemphigus 10. Photomicrograph showing acautlrolysis, specimen of pemphigus vegetans.
vegetans involving oral cavity. Tzanck cells, and a microalwcrss
in gingival
Volume Number
Pemphigus
20 4
Fig.
11. Eosinophils
Pig.
of pemphigus formation.
vulgaris
The author wishes micrographic illustrations
1% Oral
which
within
lesions
blood
vessel
in pemphigus
tends to increase
vegetans
407
in submucosa.
vegetans.
the patient’s
resistance
to express his appreciation to Mr. Henry S. Marasco for and to Mr. George Thomas for the composite drawing.
to bulla the
photo-
REFERENCES
1. Lever, 87:
W. F., and White, 12-26,
H.:
Treatment
of Pemphigus
With
Corticosteroids,
Arch.
Dermat.
1963.
2. Lever, W. F.: Pemphigus, Medicine 32: l-124, 1953. 3. Costello, M. J., Jaimovieh, L., and Dannenberg, M.: Treatment, of Pemphigus, J.A.M.A. 165: 1249-1255, 1957. 4. Director, William: Pemphigus Vegetans : Clinicopathologic Correlation, Arch. Dermat. 66: 343-352, 1952. 5. Rice, J. S., Hurt, W. C., and Rovin, S.: Pemphigus Vegetans, ORAL BURG., ORAL MED. & ORAL PATH. 16: 1383.1394,1963.
C. Hurt, GENERAL
Lieutenant HOSPITAL,
Colonel, FORT
SAM
USA HOUSTON,
TEXAS
P
emphigus vegetans is rare in this country. Therefore, it is difficult to gather material for a study of this disease. The purpose of the present article is to compare the histopathologic and clinical findings in pemphigus vegetans with those in pemphigus vulgaris, using material from the files at Brooke General Hospital and Womack Army Hospital. Pemphigus vegetans is of particular interest to dentists, since it begins in the oral cavity in more than 50 per cent of the cases. Often the oral lesions persist for months before skin lesions appear. It is during this time that the patient may consult his dentist for diagnosis and treatment. The need for early diagnosis in pemphigus has been stressed by Lever and White.l If the diagnosis is made soon after the onset, early trea.tment with corticosteroids materially improves the prognosis. According to Lever,2 there are four basic variants of pemphigus: (1) pemphigus vulgaris, (2) pemphigus vegetans, (3) pemphigus foliaceous, and (4) pemphigus erythematosus ( Senear-Usher syndrome). Of these four variants, pemphigus vegetans appears to be the most rare. Costello and associates3 reported fifty-two cases of pemphigus. Only one of the patients had pemphigus vegetans, whereas there were forty-two cases of pemphigus vulgaris, five cases of pemphigus foliaceous, and four cases of pemphigus erythematosus. More recently, Lever and White1 reported forty-six cases, only one of which was pemphigus vegetans. The histopathologic characteristics of skin lesions often pose diagnostic problems, but in pemphigus vegetans they are fairly distinctive. Perhaps Director4 has given the best interpretation of the microscopic changes. He believes that pemphigus vegetans is a reactive phase of pemphigus vulgaris which lends the patient increased resistance to the disease. In pemphigus vulgaris acantholysis, or loss of cohesion between the prickle cells, is seen. This results in the formation of cleavage vesicles within the not
The opinions or assertations to be construed as reflecting
contained the views
herein are the private ones of the author of the Department of the Army.
and
are
481
482
O.P., O.M. & 0.1’.
Ilutf
Octolwr,
1965
+dermis, just above the basal-cell layer. ‘I’hr~se vesicles Ina?- contain neutrophils and, in some instancrs, eosinophils. Also seen in these vesicles, on occasion, a?e the so-called Tzanck cells, which arc loosenetl prickle cells that, have floated free and are in various stages of degeneration (Fig. 1). The vesicles expand, forming large bullae. The rupture of these bullae results in loss of fluid, salts, and protein since, after rupture, the remaining surface cells break down, leaving large ulcerations (Fig. 2). Since the bullae cover large areas of the body surface, t,he loss of these elements and the likelihood of secondary bacttlrial invasion become serious problems.
Fig.
1. Photomicrograph
Fig.
Z. Pemphigus
of bulla
vulgaris
with
formation
extensive
in pemphigus
skin
involvement.
vulgaris.
Volume Number
20 4
Pemphigus
vegetans
483
In pemphigus vegetans the basic histopathologic changes are the same as in pemphigus vulgaris, but there are several important differences. Unlike pemphigus vulgaris, pemphigus vegetans is characterized by hyperplasia of the epidermis, especially the rete pegs or ridges. This hyperplasia, in part, prevents the breakdown of the surface epithelium. Acantholysis and vesicle formation are still seen, just as in pemphigus vulgaris (Fig. 4), but the thicker epithelium prevents spreading of the vesicle and large bulla formaCon. Fig. 3 is a composite drawing of the changes that may occur in the skin. The vesicle formed at C may be smothered by the regrowth of the hyperplastic cpithelium and carried toward the surface. Therefore, vesicles may be seen anywhere between the suprabasilar layer and the keratinized layer. As the vesicles migrate through the epithelinm, they may collect eosinophils that have wandered from the subepithelial connective tissue through the loosened epidermal cells. This results in the focal areas of eosinophils and neutrophils that are called microabscesscs(Figs. 3,B and 5). While there may be small areas of ulceration in pemphigus vegetans, the hyperplasia prevents massive exposure of underlying connective tissue and therefore keeps fluid, protein, and salts from escaping. Secondary bacterial invasion is also minimized. In the skin, the hyperplasia may throw up folds or irregular ridges (Fig. 6). Between the folds are troughs containing keratin and the contents of shedding microabscesses(Figs. 34, 6, and ‘7). These ridges are seen clinically as vegetations (Fig. 8). In the oral mucosa the hyperplasia of the rctc pegs is marked and may form a retiform pattern (Fig. 9). While actual folds are not seen in the oral mucous membrane, the surface may be rough and irregular. Acantholysis is demonstrable, and eosinophilic migration may be striking. Microabscesscscontaining eosinophils
Fig. 3. Composite drawing of histopathologic changes seen in skin affected vegetans. At C a vesicle has formed. At B a vesicle has been carried toward hyperplastic epithelium; it has collected many eosinophils and may be termed At B a microabscess is being shed.
by pemphigus the surface by a microabscess.
and perhaps a few neutrophils are part of the picture (Fig. IO). Eosinophils may be seen within the blood vessels and connective tissue of the submucosa (Fig. 11). Most authorities a.gree tha.t there is very little change: except for edema in the connective tissue underlying the affected epithelinm, in either pcmphigus vegetans or vulgaris. The histologic changes in the skin arc manifested clinically as dark brown or reddish fungoid masses (Fi,.w 8). These may u-ecp Prom the fissures and folds and then crust over. Although these vegetations do not cover t.he entire body surface, they may cover large arcas of it. The clinical lesions of the oral rnncosa often precede the skin lesions by months. The gingiva, buccal mucosa, palate, and pharynx may be involved. The oral lesions form a scrpiginous pattern throughout the mouth (Fig. 12j. The surface of these lesions resembles pus and can be wiped off with a cotton swab, leaving a bright red, weeping base that, usually does not bleed and is not
Pig.
Fig. Pig.
4.
4. Vesicle formation in skin section 5. Microabscess containing eosinophils
of pemphigus vegetans. dthin the epidermis
in pemphig,rus
rcgctans.
Volume Number
Pemphigus
20 4
cegetans
485
P
Pig.
Pig. Fig.
6. Skin seqtion of pemphigus vegetans. 7. High-power view of shedding microabscess
Fig.
8. Clinical
photograph
of vegetations
in skin
section
seen in pemphigus
of pemphigus
vegetans.
vegetans.
486
O.K. O.,\l. h 0.1’. ol~tnh’r, lRfj5
Hurt
SUMMARY
The histopathologic and clinical findings in pemphigus vegetans are compared to he a variant with those in pemphigus vulgaris. Pemphigus regeta.ns appears
Fig.
Fig. Pig.
I)iopsy
10.
9. Gingivnl biopsy specimen of pemphigus 10. Photomicrograph showing acautlrolysis, specimen of pemphigus vegetans.
vegetans involving oral cavity. Tzanck cells, and a microalwcrss
in gingival
Volume Number
Pemphigus
20 4
Fig.
11. Eosinophils
Pig.
of pemphigus formation.
vulgaris
The author wishes micrographic illustrations
1% Oral
which
within
lesions
blood
vessel
in pemphigus
tends to increase
vegetans
407
in submucosa.
vegetans.
the patient’s
resistance
to express his appreciation to Mr. Henry S. Marasco for and to Mr. George Thomas for the composite drawing.
to bulla the
photo-
REFERENCES
1. Lever, 87:
W. F., and White, 12-26,
H.:
Treatment
of Pemphigus
With
Corticosteroids,
Arch.
Dermat.
1963.
2. Lever, W. F.: Pemphigus, Medicine 32: l-124, 1953. 3. Costello, M. J., Jaimovieh, L., and Dannenberg, M.: Treatment, of Pemphigus, J.A.M.A. 165: 1249-1255, 1957. 4. Director, William: Pemphigus Vegetans : Clinicopathologic Correlation, Arch. Dermat. 66: 343-352, 1952. 5. Rice, J. S., Hurt, W. C., and Rovin, S.: Pemphigus Vegetans, ORAL BURG., ORAL MED. & ORAL PATH. 16: 1383.1394,1963.