346
J Oral Maxillofac Surg
65:346-348, 2007
Pyostomatitis Vegetans: A Clinical Marker
of Silent Ulcerative Colitis
Michael Markiewicz,* Lakshmanan Suresh, MS,†
Joseph Margarone III, DDS,‡ Alfredo Aguirre, DDS, MS,§
and Corstiaan Brass, MD储
Pyostomatitis vegetans (PV) is a rare, benign, and
chronic disorder of the oral soft tissues characterized
by the presence of multiple pustules with an erythem-
atous base.1 PV is considered a highly specific clinical
marker for inflammatory bowel disease (IBD).2,3 The
first reports of PV were documented by Hallopeau in
1898, who described 2 cases with unusual pustular
dermatosis and oral lesions that he called pyodermite
vegetante.4 The term pyostomatitis vegetans was coined
by McCarthy5 in 1949, who believed that it was a variant
of pyodermite vegetante. Since McCarthy’s report in
1949, approximately 50 cases have been published in
the literature.4,6 In this report, we present a typical
case of PV.
Report of a Case
A 30-year-old white male was referred with a chief com-
plaint of “swelling inside of the cheeks.” The patient had
developed painless swellings 6 months earlier that have
been progressively increasing in size and prompted him to
seek medical care. The primary care physician referred him
to his general dentist, who suspected a fungal infection and
prescribed clotrimazole. The lesions did not respond to the
antifungal therapy. Since then he had seen an otolaryngol-
ogist, a dermatologist, and an infectious disease specialist
Received from the State University of New York at Buffalo, Buffalo,
NY.
*Junior Dental Student, School of Dental Medicine.
†Junior Dental Student, School of Dental Medicine.
‡Clinical Assistant Professor, Department of Oral and Maxillofa-
cial Surgery, School of Dental Medicine.
§Director, Advanced Oral and Maxillofacial Pathology; and Pro-
fessor, Department of Oral Diagnostic Sciences, School of Dental
Medicine.
储Clinical Associate Professor, School of Medicine.
Address correspondence and reprint requests to Dr Aguirre:
Oral Diagnostic Sciences, 355 Squire Hall, School of Dental Medi-
cine, State University of New York at Buffalo, Buffalo, NY 14214-
8006; e-mail: aguirr@buffalo.edu
© 2007 American Association of Oral and Maxillofacial Surgeons
0278-2391/07/6502-0034$32.00/0
doi:10.1016/j.joms.2005.07.020
PYOSTOMATITIS VEGETANS
without any success. When we saw the patient on October
10, 2002, his past medical history was significant for ulcer-
ative colitis (UC) that was diagnosed in 1992 and was being
treated with mesalamine (1.6 g 3 times a day). The patient
denied tobacco habits. Clinical examination showed bilat-
eral swelling and thickening of the buccal mucosa approx-
imately 0.5 cm from the lip commissures and extending
posteriorly to nearly the entire buccal mucosal surfaces (Fig
1). The tissue was pink and had a pebbled surface with
multiple deep fissures and moderate induration. Incisional
biopsy of the right and the left buccal mucosa was carried
out under local anesthesia and the specimens were submit-
ted for histopathological examination. Microscopic exami-
nation revealed a stratified squamous epithelium showing
pseudoepitheliomatous hyperplasia with intraepithelial mi-
croabscesses comprised of eosinophils and neutrophils. In-
traepithelial clefting was also noted. The papillary lamina
propria displayed an acute and chronic inflammatory cell
infiltrate with prominent eosinophils and perivascular in-
flammation. The histopathogical features were suggestive of
pyostomatitis vegetans (Figs 2, 3). Additional direct immu-
nofluorescence testing for IgG, IgA, IgM, fibrin, and com-
plement component C3 were negative and ruled out pem-
phigus vulgaris. A recommendation to evaluate the patient
for active inflammatory bowel disease was made.
The patient underwent a colonoscopy on October 21,
2002, which showed a new and active region of ulcerative
colitis in the cecum and cecal floor polyps/pseudopolyps.
Balsalazide disodium (2.25 g orally 3 times a day) was
prescribed and resulted in a vast improvement of the pa-
tient’s gastrointestinal complaints. A follow-up examination
on December 5, 2002, showed complete resolution of the
oral lesions and remission of the IBD.
Discussion
Pyostomatitis vegetans (PV) is a rare, benign chronic
disease characterized by the presence of multiple oral
pustules, erosions, and vegetating dermatosis of the
skin.1,4,5 PV is rare and may affect any age group, but
is most common in young and middle-aged adults.
There is a significant male predilection with a male to
female ratio of 3:1.7 The pathogenesis of PV is poorly
understood. A search for an infectious etiology has
persistently yielded negative results, for pathogenic
bacteria, viruses, and fungi. Cultures have consis-
tently shown normal oral flora.4
MARKIEWICZ ET AL
FIGURE 1. Left buccal mucosa showing typical edematous appear-
ance with background erythema and “snail track” ulcerations. The right
buccal mucosa presented identical clinical features.
Markiewicz et al. Pyostomatitis Vegetans. J Oral Maxillofac Surg
2007.
Sometimes, cutaneous and oral lesions may coexist.
The dermal lesions are typically asymmetrical and
usually affect the axillary folds and groin and, to a
lesser extent, involve the face and the scalp.8,9 Al-
though any area of the mouth can be affected, the
most common sites of occurrence are the labial and
buccal mucosa, followed by the hard and soft palate,
gingiva, and sulci.7 The floor of the mouth is less com-
monly affected and the tongue is usually spared.4,10 The
oral lesions consist of friable pustules with an ery-
FIGURE 2. Low magnification demonstrating the presence of intra-
epithelial clefting and an admixture of acute and chronic inflammatory
cells with prominent eosinophils (H&E; original magnification ⫻10).
Markiewicz et al. Pyostomatitis Vegetans. J Oral Maxillofac Surg
2007.
347
FIGURE 3. High magnification of intraepithelial eosinophils amidst
acantholytic keratinocytes (H&E; original magnification ⫻400).
Markiewicz et al. Pyostomatitis Vegetans. J Oral Maxillofac Surg
2007.
thematous base that erodes, coalesces and undergoes
necrosis to form a typical “snail tracks” appearance.4
Patients usually experience no pain or discomfort
despite extensive involvement of the oral tissues.10
Peripheral eosinophilia has been reported in 90% of
the cases and can aid in the diagnosis.4,8 Liver dys-
function has been reported to be associated with
PV.11,12 Philpot et al3 reported that 21% of all PV cases
have had some type of liver dysfunction, thus, pa-
tients with PV should be evaluated for hepatic disor-
ders.
Histological examination of the oral lesions
shows characteristic intraepithelial and/or subepi-
thelial microabscesses containing numerous eosin-
ophils.12 As the lesion matures there are fewer
eosinophils. The underlying lamina propria con-
tains a dense mixed inflammatory infiltrate contain-
ing eosinophils, neutrophils, lymphocytes, and
plasma cells. Acanthosis, hyperkeratosis, and areas
of intraepithelial dissociation suggestive of acan-
tholysis are also evidenced.12 Direct immunofluo-
rescence (DIF) in PV is usually negative or may
render weakly positive and/or inconclusive results
that help to distinguish it from vesiculobullous dis-
eases such as pemphigus vulgaris.4 Our patient had
intraepithelial microabscesses containing eosino-
phils and neutrophils, and the DIF studies were
negative for deposits of IgA, IgG, and C3.
Management of PV is difficult. Treatment is often
based on treating the underlying gastrointestinal
disease via diet modifications, and the administration
of systemic agents such as antispasmodics, antibiotics,
sulfalazine, corticosteroids, azathioprine, and dapsone.
Surgical treatment in severe cases of IBD involves
total colectomy and has resulted in permanent remis-
348
sion of symptoms.4 The oral lesions can be managed
with antiseptic mouthwashes such as chlorhexidine
or topical corticosteroids mouthwash.13 However,
topical steroid therapy has limited success and complete
resolution of the lesions usually requires the adminis-
tration of systemic steroids such as prednisolone. Al-
ternatively, dapsone,2 azathioprine,14 and sulfame-
thoxypyridiazine15 have been effectively used as an
alternative to steroid therapy. The use of dapsone is
limited by its side effects, which include hemolytic ane-
mia, hepatitis, agranulocytosis, and the possibility of a
drug-mediated allergic reaction. Sulfamethoxypyridi-
azine has the advantage of being both effective and
less apt to produce erythema multiforme and hemo-
lytic anemia.15 Azathioprine is only minimally effec-
tive and is associated with the risk of bone marrow
suppression and hepatotoxicity.
In summary, we have presented a patient with a
history of ulcerative colitis in remission who later
developed PV that correlated with the emergence of de
novo, asymptomatic inflammatory lesions of the colon.
PV appears to be a reliable clinical marker for active IBD.
Balsalazide disodium was effective to successfully man-
age the PV and ulcerative colitis of this patient.
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