Dental Science - Case Report

Oral Crohn’s disease without intestinal

manifestations
Gingisetty Harikishan, Nagate Raghavendra Reddy, Harikrishnan Prasad1,
Subappa Anitha

Departments of
Periodontia and 1Oral
Pathology, KSR Institute
of Dental Science and
Research, KSR Kalvi
Nagar, Thokkavadi,
Tiruchengode,
Tamil Nadu, India
Address for correspondence:
Dr. Gingisetty Harikishan
E-mail: hari2869@gmail.com
Received : 01-12-11
Review completed : 02-01-12
Accepted : 26-01-12
ABSTRACT
Crohn’s disease is a granulomatous inflammatory bowel disease and was described in 1932 as a chronic
granulomatous disorder of the terminal ileum and is now considered a distinct member of the inflammatory
bowel disease family. It may affect any part of the gastrointestinal tract. Oral Crohn’s disease has been reported
frequently in the last three decades with or without intestinal manifestations. In the latter case, it is considered
as one of the orofacial granulomatosis. There has been much doubt whether intestinal manifestations of Crohn’s
disease will eventually develop in the orofacial granulomatosis. We present a female patient aged 22 years with
prominent clinical findings such as persistent swelling of lower and upper lip with fissuring and angular cheilitis,
granulomatous gingival enlargement, and cobblestone or corrugated appearance of labial mucosa, which are
suggestive of Crohn’s disease, but with no evidence of other gastrointestinal involvement. The patient underwent
surgical treatment with external gingivectomy procedure. A 6-month follow-up showed minimal recurrence.
KEY WORDS: Crohn’s disease, orofacial granulomatosis, gingival enlargement

C rohn’s disease is a chronic granulomatous inflammatory

bowel disease characterized by localized areas of non-
specific, non-caseating granulomas. Among the chronic forms
of inflammatory bowel diseases, it can be differentiated on the
basis of histology, clinical presentation, laboratory investigations,
and disease progression. Although considerable data are
available regarding the systemic features of Crohn’s disease, data
concerning the oral and dental findings are limited and show that
9% of patients with Crohn’s disease have oral manifestations.[1]
The oral findings associated with Crohn’s disease were initially
described in 1969, first by Dyes et al. detailing two chronic
disease patients[2] and later by Issa in 1971.[1]
Oral Features of Crohn’s Disease[2-4]
1. Persistent, diffuse soft tissue swelling of lips and buccal
mucosa, which is edematous or firm on palpation. Lip
involvement can lead to vertical fissuring.
2. Cobblestone or corrugated appearance of buccal or labial
mucosa.
3. Linear aphthous ulcerations.
4. Angular cheilitis.
5. Granular, edematous, hyperplastic enlargement of gingival,
involving attached gingiva and extending up to mucogingival
line with or without ulcerations.
6. Persistent lymphadenopathy of submandibular lymph nodes
which are firm, occasionally tender to painful.
7. Epithelial tags or folds (fibroepithelial hyperplasia) seen on
buccal mucosa, vestibule, or retromolar areas.
Extraintestinal manifestations of Crohn’s disease have been
recognized, mainly pyoderma gangrenosum or erythema
nodosum. Skin lesions seem to be especially common. Verbov
in 1973 reported 106 cases of Crohn’s disease, 85% of which
had skin manifestations.[1]

Access this article online We hereby report a case of oral Crohn’s disease without
Quick Response Code:
intestinal manifestations and with typical intraoral findings
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Case Report
DOI:
10.4103/0975-7406.100322 A 22-year-old female of Indian origin was referred to the
Department of Periodontics, with a complaint of persistent

How to cite this article: Harikishan G, Reddy NR, Prasad H, Anitha S. Oral Crohn's disease without intestinal manifestations.
J Pharm Bioall Sci 2012;4:431-4.

Journal of Pharmacy and Bioallied Sciences Vol 4 August 2012 Supplement 2 - Part 4 S431 
AQ5  Harikishan, et al.: Oral Crohn’s disease
swelling of the lower lip with recurrent episodes. Extraoral
findings revealed mild angular cheilitis and the lower lip
was greatly swollen with marked vertical fissuring [Figure 1].
Intraoral findings revealed generalized granulomatous gingival
hyperplasia, extending up to the mucogingival line and which was
markedly seen on facial aspect covering almost the entire length
of clinical crowns [Figures 2–4]. On palpation, gingiva was tender.
There was marked cobblestone appearance of labial mucosa on
the right side of lower lip which was tender on palpation. The
right and left submandibular lymph nodes were palpable and
tender. Family and medical history were not significant. Patient
did not give any history of gastrointestinal disturbances.
Laboratory investigations
Hematological examination was performed and results were
within normal limits. However, the peripheral blood smear
showed evidence of microcytic hypochromic anemia. Further
blood investigations showed low serum iron and folate levels
and raised erythrocyte sedimentation rate.
Mantoux test and Kveim test proved negative, which ruled
out active tubercular infection and sarcoidosis. Biopsy was also
performed to rule out intestinal involvement.
Intraoral radiological examination revealed normal radiological
Figure 1: Marked vertical fissuring seen in the lower lip
Figure 3: Gingival hyperplasia in the left anterior region
 S432 Journal of Pharmacy and Bioallied Sciences Vol 4 August 2012 Supplement 2 - Part 4
features with mild loss of interdental bone in relation to the
mandibular posterior teeth, which is common in this age group.
Histopathologic examination
On histopathologic examination [Figure 5], the lesion was
seen to be covered by stratified squamous parakeratinized
epithelium, which varied from atrophic to hyperplastic in
areas. Areas of spongiosis and ulceration were also evident.
Connective tissue was fibrocellular and edematous, moderately
infiltrated by chronic inflammatory cells, chiefly lymphocytes
and macrophages. Blood vessels, areas of hemorrhage, and
isolated multiple non-caseating granulomas specifically located
subepithelially were also seen.
Each non-caseating granuloma was loosely textured and
consisted of a central mass of epithelioid cells surrounded by
chronic inflammatory cells and occasional foreign body type or
Langhan’s type of giant cells.
Diagnosis
Based on our clinical findings, histopathologic examination, and
results of laboratory investigations, we arrived at a diagnosis of
oral Crohn’s disease without intestinal manifestations.
Figure 2: Gingival hyperplasia in the right lateral incisor-canine region
Figure 4: Marked erythema seen in the affected gingiva
 Harikishan, et al.: Oral Crohn’s disease 

Treatment

After performing possible investigations, our treatment

depended mainly on anti-inflammatory drugs and corticosteroid
supplements. Topical steroid applications were advised
for painful oral ulcerations. The angular cheilitis and oral
ulcerations responded to the use of 1% hydrocortisone ointment
and vitamin supplements. External gingivectomy was planned
quadrant-wise after thorough oral prophylaxis and sub gingival
scaling.

The patient was followed up for approximately 15 months

during which time no gastrointestinal symptoms were noticed.
A repeat barium meal and follow-up were made. Repeated
hematological tests showed no marked variation from the
Figure 5: Non caseating granuloma seen in the connective tissue
initial findings.

The clinical outcome and improvement in this patient was

not satisfactory. Normal appearance of the gingiva was not
achieved even though there was dramatic improvement with
oral ulcerations and lip swelling [Figures 6 and 7].

Discussion

The underlying granulomatous inflammation of Crohn’s

disease can involve any segment of the gastrointestinal tract.
While the etiology is currently unknown, immunological
dysfunction may play a major role in disease development.
However, it is difficult to distinguish between oral Crohn’s
disease and other types of orofacial granulomatoses.[5,6] At the
present time, however, the diagnosis is essentially a clinical one
and reinforced by histopathologic study of tissue removed at Figure 6: Post-operative extraoral appearance
biopsy. Sigmoidoscopy alone does not eliminate the possibility
of intestinal disease and a biopsy is essential because positive
findings have been reported in cases with normal sigmoidoscope
appearance.

Numerous investigators have proposed a prominent role of

altered host immunity in the pathogenesis of Crohn’s disease.
Reduced mucosal barrier factors and increased intestinal
permeability was reported, yet follow-up studies have failed
to confirm this finding. Examination of neutrophil functions
has yielded conflicting results including reduced migration,
chemotaxis, and superoxide anion production. Inhibitors
to both chemotactic factors and leukocyte chemotaxis have
been detected in the sera of patients with Crohn’s disease and
ulcerative colitis.[7] More recently, circulating interleukin-6
(IL- 6) levels were found to be significantly elevated among
Crohn’s disease patients, regardless of anti-inflammatory
medication.[8] Figure 7: Intraoral post-operative appearance

Nutritional or dietary factors such as a negative history of breast
feeding, increased sugar intake, and increased ingestion of food
additives or chemicals reportedly can contribute to an increased
incidence of Crohn’s disease.[9]
Microscopically, the initial lesion starts as a focal inflammatory
infiltrate around the intestinal crypts, followed by ulceration of
superficial mucosa. Later, inflammatory cells invade the deep
mucosal layers and, in that process, begin to organize into non-
caseating granulomas. The granulomas extend through all the
layers of the intestinal wall and into the mesentery and the
regional lymph nodes. Neutrophil infiltration into the crypts
forms crypt abscesses, leading to destruction of the crypt and
atrophy of the colon. Chronic damage may be seen in the form

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 Harikishan, et al.: Oral Crohn’s disease
of villous blunting in the small intestine as well. Ulcerations are
common and are often seen in a background of normal mucosa.
Although granuloma formation is pathognomonic of Crohn’s
disease, its absence does not exclude the diagnosis.[10] Tooth
pastes containing silica and silicates that are capable of inducing
granuloma formation can increase the incidence of Crohn’s
disease. To date, however, scientific evidence concerning this
hypothesis is limited and inconclusive.[11]
In the absence of intestinal manifestations, the diagnosis of
oral Crohn’s disease usually depends on clinical appearance
together with histological findings of non-caseating epithelioid
granulomas. Histologically, however, it is difficult to distinguish
between oral Crohn’s disease and other types of granulomatoses.
The latter is a generalized term used to describe a group of
chronic disorders of unknown etiology characterized by facial
and lip swellings, gingival enlargements, oral ulcerations, and
a history of facial nerve paralysis in some cases.
The association of severe progressive periodontal destruction
with Crohn’s disease was reported by Lamster et al. in 1978. Yet,
in 1982, Lamster et al. detected ‘‘overt oral disease” in only 2 of
10 inflammatory bowel disease patients with no direct reference
to periodontal disease status. But in our patient, there was no
detectable periodontal involvement. In 1988, Vandyke et al.
reported 20 inflammatory bowel disease patients, of whom only
9 patients showed periodontal diseases and altered neutrophil
function.[12]
Controversy continues about whether intestinal Crohn’s disease
will eventually develop in some of these patients with only oral
manifestations and, if so, in how many patients and after how
long. The answer to these questions is not clearly resolved.
In our patient, intra- and extraoral manifestations, along with
histopathologic findings, were similar to those of Crohn’s
 S434 Journal of Pharmacy and Bioallied Sciences Vol 4 August 2012 Supplement 2 - Part 4
disease, without intestinal manifestations. Ghandour et al.
stated that the intestinal manifestations can appear as late as
9 years after the oral lesions.[1] A 15-month follow-up period of
our patient showed minimal recurrence of the gingival lesions.
Extraoral healing was satisfactory. However, long-term follow-
up of these patients would be wise. In the case of abdominal
symptoms, investigations should be repeated.
References
1. Ghandour K, Issa M. Oral Crohn’s disease with late intestinal
manifestations. Oral Surg Oral Med Oral Pathol 1991;72:565-7.
2. Kalmar JR. Crohn’s disease: Orofacial considerations and disease
pathogenesis. Periodontology 2000 1994;6:101-15.
3. Tyldesley WR. Oral Crohn’s disease and related conditions. Br J Oral
Surg 1979;17:1-9.
4. Field EA, Tyldesley WR. Oral Crohn’s disease revisited-a 10-year-
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5. Shafer WG, Hine MK, Levy BM. Editors. A text book of oral pathology.
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6. Peter C, Reade, Bryan G, Radden. Non-microbial inflammatory
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7. Rhodes JM, Potter BJ, Brown DJ, Jewel DP. Serum inhibitors of
leukocyte chemotaxis in Crohn’s disease and ulcerative colitis.
Gastroenterology 1982;82:1327-37.
8. Gross V, Andus T, Caesar I, Rotu M, Scholmerich J. Evidence for
continuous stimulation of interleukin - 6 Production in Crohn’s
disease, Gastroenterology 1992;102:514-19.
9. Katschinski B, Logan RF, Edmond M, Lagman MJ. Smoking and sugar
intake are separate but interactive risk factors in Crohn’s disease.
Gut 1988;29:1202-6.
10. Thoreson R, Cullen JJ. Pathophysiology of inflammatory bowel
disease: An overview. Surg Clin North Am 2007;87:575-85.
11. Mayberry JF, Rhodes J, Newcombe RG. Breakfast and dietary aspects
of Crohn’s disease. Br Med J 1978;2:1401-5.
12. Lamster 1, Souis S, Hannigan A, Kolodkin A. An association between
Crohn’s disease, periodontal disease and enhanced neutrophil
function. J. Periodontal 1978;49:475-9.
Source of Support: Nil, Conflict of Interest: None declared.
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