RARE CLINICAL ENTITY - IDIOPATHIC

OROFACIAL GRANULOMATOSIS

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ABSTRACT

Orofacial granulomatosis (OFG), a noncaseating granulomatous

inflammation is a rare disorder affecting the orofacial region

which is clinically characterized by diffuse, nontender, soft to

firm enlargement involving lip, tongue, gingiva, buccal mucosa,

floor of the mouth and other intra-oral sites. The term Idiopathic

Orofacial granulomatosis refers to conditions restricted to the

oral region without any identifiable systemic granulomatous

diseases. Though certain food and food additives, dental materials

and various microbiological agents may be possible etiological

agents, the definite cause has yet to be identified. The exact antigen

inducing the immunological reaction differs in individuals.

Evidence for the role of genetic predisposition to the disease is

sporadic. Delayed type of hypersensitivity reaction appears to

play a significant role. Early diagnosis and management prevent

the systemic complications. Multidisciplinary approaches are

evolved to achieve periodontal esthetics. This case report entails

the overview of enlargement of lip and gingiva in a 28year old

female of 2 years duration without any identifiable etiology and

was treated by gingivectomy.

KEYWORDS:

Orofacial granulomatosis [OFG], Gingival

enlargement, Corticosteroid therapy

JIDAM/Chennai/Volume:7/Issue:4/Pages 152 - 156/Oct-Dec 2020

152

Address for Correspondence:

Dr. Sangeetha.S.
Postgraduate Student, Department
of Periodontology, Tamilnadu
Government Dental College,
Muthusamy Salai, George Town
Chennai-600003, Tamilnadu, India.
Email id: sangeethadoc1190@gmail.
com

Received

: 16.11.2020

Accepted

: 15.12.2020

Published

: 27.12.2020

CASE REPORT

Dr. Jaishree Tukaram Kshirsagar, Dr. Sangeetha.S

Department of Periodontology,

Tamilnadu Government Dental College, Chennai, Tamilnadu, India.

DOI:1.37841/jidam_2020_V7_I4_04

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Sangeetha et al: Idiopathic Orofacial Granulomatosis

153

INTRODUCTION :

Melkersson first described a case of occurrence

of granulomas in the orofacial region with facial

palsy and orofacial oedema without a recognized

underlying systemic condition in 1928

. Wiesenfield

proposed the term orofacial granulomatosis in 1985.

Orofacial granulomatosis is a rare, idiopathic clinical

entity causing swelling of soft tissues of the oral and

maxillofacial region, with the histological evidence

of noncaseating granulomatous inflammation in the

absence of diagnosable systemic Crohn’s disease or

sarcoidosis. It also includes Melkersson Rosenthal

syndrome and cheilitis granulomatosa. It is clinically

characterized by diffuse, nontender, soft to firm,

painless extra-oral swelling restricted to one or both

lips and intraoral sites involving tongue, gingiva and

buccal mucosa

2, 3

. Though several theories including

infection, genetic, and allergy have been proposed,

the exact etiology has still not been established

4 –

. Systemic and local causes must be excluded as

the clinical and histological features of OFG which

has close similarities with the manifestations of few

systemic diseases such as tuberculosis, sarcoidosis,

Crohn’s disease, angioedema and local causes

such as foreign body reaction and allergy

. Early

diagnosis, treatment and monitoring of cases prevent

the development of fatal systemic diseases.

CASE REPORT:

A 26-year-old female patient reported to the

Department of Periodontics with the chief complaint

of intermittent, painless swelling of lips and gums for

the past 2 years. Patient’s past dental, medical, drug

history and history of allergy were non-contributory.

Extra oral examination and palpation showed

diffuse, soft, nontender, upper and lower lip swelling

of normal temperature (Fig. 1). Mild fissures on

the vermilion border of upper and lower lips was

present. On intraoral examination, diffuse gingival

enlargement extending from 17 to 27 up to the

mucogingival junction in the maxilla and involving

43 to 33 in mandible up to attached gingiva was

seen. The enlargement extended coronally almost

up to the incisal margins in the maxilla and up to

the middle third in the mandible. The gingiva was

pale pink in color, smooth and shiny with absence of

stippling. Interdental spacing was seen where none

existed before and plaque and calculus formation

was seen. Bleeding on probing was elicited. There

were no considerable changes on the dorsal surface

of the tongue. Orthopantomogram did not show

alveolar bone loss in the maxillary and mandibular

region. (Fig:2) Investigations were made, red blood

cells count, hemoglobin, hematocrit, total leukocyte

count, differential leukocyte count, platelets count,

hematocrit/ packed cell volume, mean corpuscular

volume, mean corpuscular hemoglobin, mean

corpuscular hemoglobin concentration and serum

angiotensin convertase enzyme, chest radiograph

and mantoux tests were advised. All blood and

serum investigations were within normal limits.

Chest radiograph was normal, and the mantoux test

was also negative. History and clinical examination,

aided us to make the provisional diagnosis of

orofacial granulomatosis of the upper and lower lip

with maxillary and mandibular gingival enlargement.

After the establishment of the provisional

diagnosis, intra-lesional injection of triamcinolone

(40 mg/mL) was injected weekly into each lateral

edge of the upper and lower lip till 3 weeks. Upper

and lower lip was anesthetized by infraorbital and

mental nerve block to reduce the pain and discomfort

arising after the intra-lesional injection. Thereafter,

gingival enlargement was initially treated in phase

I therapy including scaling and root planing. After

phase I therapy, the gingival tissue become firmer

due to the reduction of edematous component of the

enlargement. Then, gingivectomy was planned for

maxillary region. A written informed consent was

obtained. After administration of local anesthesia

to the affected area, the depths of the pockets are

assessed using a periodontal probe and bleeding

points are marked. The bleeding points are used as

a guide for incision and to determine the depth of

the tissue to be excised. Gingivectomy was done for

maxillary region and periodontal dressing was placed

for 1week (Fig:3) Histopathological examination of

the excised tissue showed the presence of stratified

squamous epithelial lining with acanthosis and

scattered chronic granulomatous inflammation in

the underlying fibro-collagenous stroma (Fig: 4)

The case was followed for one year without any

recurrence (Fig:5)

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Sangeetha et al: Idiopathic Orofacial Granulomatosis

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Fig 1: Preoperative photograph showing upper and

lower lip swelling and maxillary and mandibular

gingival enlargement

Fig 2: Orthopantomogram X-ray showing no alveolar

bone loss

Fig 3: Gingivectomy performed in maxillary region

Fig 4: Stratified squamous epithelial lining with

acanthosis and scattered chronic granulomatous

inflammation in underlying fibro-collagenous stroma

Fig 5: Postoperative photograph showing reduced

upper and lower lip swelling and maxillary and

mandibular gingival enlargement after 1 year

DISCUSSION:

The diagnosis of orofacial granulomatosis

is made by histological presence of noncaseating

granulomas with the clinical presence of orofacial

swellings. Other conditions for granulomatous

formation like sarcoidosis, Tuberculosis, Crohn’s

disease, angioedema, Melkersson – Rosenthal

syndrome are excluded by appropriate clinical

and laboratory investigations. The sarcoidosis

was elicited on the basis of chest radiograph and

serum angiotensin converting enzyme levels.

Crohn’s disease was ruled out on the absence of

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Sangeetha et al: Idiopathic Orofacial Granulomatosis

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steroid therapy with surgical excision.

CONCLUSION:

Orofacial granulomatosis is arduous to

diagnose clinically because of varied clinical

manifestations and rare occurrence. Histopathology

remains the most definitive way to diagnose this

condition. Due to lack of evidence in the literature, it

is difficult to relate a reference treatment of orofacial

granulomatosis. For evidence-based diagnosis

with detection of specific cellular markers remains

as beneficial which results in targeted therapy for

underlying pathology.

FINANCIAL SUPPORT AND SPONSORSHIP:

Nil.

CONFLICTS OF INTEREST:

There are no conflicts of interest.

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