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Javadzadeh et. al Approach to orofacial granulomatosis and review . . .

evaluation. Therefore, this term is not clinically Crohn’s disease, sarcoidosis, etc. Recently, ga-
relevant [16]. strointestinal involvement in non-endemic re-
Mucosal swellings: Buccal and labial mucosae gions such as southern Europe, Asia and devel-
may be swollen producing plications with a cob- oping countries is increasing. There is a possibil-
blestone appearance that often involves posterior ity that the prevalence of orofacial granulomato-
parts of buccal mucosa [17,18] sis which has a slight predilection to appear in
Mucosal tags: These painless tags of mucosa women increases and manifest primarily in
which are often produced at the depth of labial children and young adults [9,12,19,22].
or buccal vestibule, retromolar area or around Etiology and pathogenesis: The exact cause of the
chronic ulcerations are orange or red in color orofacial granulomatosis is currently unknown
[17,18] and has been a matter of debate for long. Five
Gingival overgrowths: Overgrowths of the free or etiologic factors can be attributed to the orofacial
attached gingiva can occur locally or diffusely. granulomatosis [23-27]:
They can precede facial or mucosal manifesta- Genetic predisposition
tions. The gingiva appears granular with normal Food allergy
pink to red in color and rarely ulcerated [19]. Allergy to dental materials
Fissured tongue Infection
The dorsal surface of the tongue may be fissured Immunologic causes
[18] Genetic predisposition: A comprehensive review
Facial nerve palsy: Paralysis of the facial motor of the literature does not show evidence to sup-
nerve may occur rarely in orofacial granulomatosis. port genetic causes for orofacial granulomatosis.
This condition can also occur as a result of formation In a study genetic factors contributed in only
of granuloma within the nerve trunk. Facial nerve 23% of cases and in another study in 6 out of 42
palsy accompanied with fissured tongue and labial cases [28] Also, it was reported in a study that
swelling is indicative of Melkersson-Rosenthal Syn- 10% of normal population could have orofacial
drome [11-20] granulomatosis, an issue that underscored the
Facial erythema and swelling: Recurrent facial role of genetic factors [29]. Association of
swelling may occur especially in genial, zygo- orofacial granulomatosis with HLA has also
matic, peri-orbital and palpebral areas of the face been studied, but authors failed to establish a
and can be unaccompanied with hypertrophy of strong correlation between HLA and pathogene-
the lips in rare occasions. These swellings are sis of orofacial granulomatosis [30,31]. Only one
non-pitting on pressure and usually are firm in study reported a significant interrelationship
palpation with an erythematous surface [17]. with HLA and orofacial granulomatosis [31].
Cervical lymphadenopathy: Patients with severe Food allergy: Orofacial granulomatosis can occur
orofacial granulomatosis can have cervical lym- because of several nutritional additives and ma-
phadenopathy that can be localized or genera- terials. Antigenic stimulants that cause delayed
lized, tender or non-tender with variable sizes hypersensitivity reactions have been associated
and usually a rubbery consistency [21]. with more than 60% of patients with orofacial
Epidemiology: Orofacial granulomatosis has been granulomatosis. It has been declared in several
defined for 90 years as a chronic persistent swel- studies that different daily nutrients such as
ling of the lip(s) with or without facial swelling chocolates, carmosine, eggs, peanuts, cinnamon,
and/or with oral and gingival mucosal enlarge- toothpastes, monosodium glutamate, alpha-
ment without any evidence of involvement in lactobumin, benzoic acid, and cocoa were initia-
other parts of the body. Occasionally involve- tors of clinical manifestations in patients with
ment of other organs has led to the diagnosis of orofacial granulomatosis [23-26,33-27]

Summer 2012; Vol. 24, No. 3 113