Orofacial granulomatosis

Orofacial granulomatosis
Classification and external resources
Specialty gastroenterology
ICD-10 K13.0 (ILDS K13.052)
ICD-9-CM 351.8
DiseasesDB 33784

Orofacial granulomatosis (abbreviated to OFG, and also termed granulomatous cheilitis, cheilitis granulomatosa, cheilitis granulomatosis, and oral granulomatosis), is a condition characterized by persistent enlargement of the soft tissues of the mouth, lips and the area around the mouth on the face. The enlargement does not cause any pain,[1] but the best treatment and the prognosis are uncertain.[2] The mechanism of the enlargement is granulomatous inflammation.[3] The underlying cause of the condition is not completely understood, and there is disagreement as to how it relates to Crohn's disease and sarcoidosis.[3]

Classification

OFG could be classified as a type of cheilitis (lip inflammation), hence the alternative names for the condition using the word cheilitis, and a granulomatous condition.

Signs and symptoms

Signs and symptoms may include:

The enlargement of the tissues of the mouth, lips and face seen in OFG is painless.[1] Melkersson-Rosenthal syndrome is where OFG occurs with fissured tongue and paralysis of the facial nerve.[1] The cause of the facial paralysis is thought to be caused by the formation of granulomas in the facial nerve, which supplies the muscles of facial expression.[2]

Causes

The cause of the condition is unknown.[6][4] The disease is characterized by non-caseating granulomatous inflammation.[3] That is, the granulomas do not undergo the caseating ("cheese-like") necrosis typical of the granulomas of tuberculosis.

There is disagreement as to whether OFG represents an early form of Crohn's disease or sarcoidosis, or whether it is a distinct, but similar clinical entity.[3][7] Crohn's disease can affect any part of gastrointestinal tract, from mouth to anus. When it involves the mouth alone, some authors refer to this as "oral Crohn's disease", distinguishing it from OFG, and others suggest that OFG is the same condition as Crohn's disease when it presents in the oral cavity.

OFG may represent a delayed hypersensitivity reaction, but the causative antigen(s) is not identified or varies form one individual to the next. Suspected sources of antigens include metals, e.g. cobalt, or additives and preservatives in foods, including benzoates, benzoic acid, cinnamaldehyde, metabisulfates, butylated hydroxyanisole, dodecyl gallate, tartrazine, or menthol,[4][6][8] Examples of foods which may contain these substances include margarine, cinnamon, eggs, chocolate or peppermint oil.[4][6][9]

Some suggest that infection with atypical mycobacteria could be involved, (paratuberculosis),[4] and that OFG is a reaction to mycobacterial stress protein mSP65 acting as an antigen.[6]

In response to an antigen, a chronic, submucosal, T cell mediated inflammatory response occurs, which involves cytokines (e.g. tumor necrosis factor alpha), protease-activated receptors, matrix metalloproteinases and cyclooxygenases.[6] The granulomas in OFG form in the lamina propria, and may form adjacent to or within lymphatic vessels.[8] This is thought to cause obstruction of lymphatic drainage and lymphedema which is manifest as swelling clinically.[6]

There may be a genetic predisposition to the condition.[6] People who develop OFG often have a history of atopy, such as childhood asthma or eczema.[4]

Diagnosis

The diagnosis is usually made by tissue biopsy, however this cannot reliably distinguish between the granulomas of OFG and those of Crohn's disease or sarcoidosis.[8] Other causes of granulomatous inflammation are ruled out, such as sarcoidosis, Crohn's disease, allergic or foreign body reactions and mycobacterial infections.[1]

Treatment

Anti-tumour necrosis factor α antagonists (e.g. infliximab) [10]

Dietary restriction of a particular suspected or proven antigen may be involved in the management of OFG, such as cinnamon or benzoate-free diets.[8]

Epidemiology

OFG is uncommon, but the incidence is increasing.[2] The disease usually presents in adolescence or young adulthood.[6] It may occur in either sex, but males are slightly more commonly affected.[6]

History

OFG was first described in 1985.[3]

References

  1. 1 2 3 4 Treister NS, Bruch JM (2010). Clinical oral medicine and pathology. New York: Humana Press. pp. 51,147. ISBN 978-1-60327-519-4.
  2. 1 2 3 4 5 6 7 8 9 10 11 Leão, JC; Hodgson, T; Scully, C; Porter, S (Nov 15, 2004). "Review article: orofacial granulomatosis.". Alimentary pharmacology & therapeutics 20 (10): 1019–27. doi:10.1111/j.1365-2036.2004.02205.x. PMID 15569103.
  3. 1 2 3 4 5 Grave, B; McCullough, M; Wiesenfeld, D (January 2009). "Orofacial granulomatosis--a 20-year review.". Oral diseases 15 (1): 46–51. doi:10.1111/j.1601-0825.2008.01500.x. PMID 19076470.
  4. 1 2 3 4 5 6 Jordan, Michael A.O. Lewis, Richard C.K. (2012). Oral medicine (Second ed.). London: Manson Publishing. pp. 128–129. ISBN 978-1840761818.
  5. ↑ Welbury R; Duggal M; Hosey MT (2012). Paediatric dentistry (4th ed.). Oxford: Oxford University Press. p. 319. ISBN 978-0199574919.
  6. 1 2 3 4 5 6 7 8 9 Scully C (2013). Oral and maxillofacial medicine : the basis of diagnosis and treatment (3rd ed.). Edinburgh: Churchill Livingstone. pp. 298–301. ISBN 9780702049484.
  7. ↑ Zbar, AP; Ben-Horin, S; Beer-Gabel, M; Eliakim, R (March 2012). "Oral Crohn's disease: is it a separable disease from orofacial granulomatosis? A review.". Journal of Crohn's & colitis 6 (2): 135–42. doi:10.1016/j.crohns.2011.07.001. PMID 22325167.
  8. 1 2 3 4 Scully, Crispian; Oslei Paes de Almeida; Jose Bagan; Pedro Diz Dios; Adalberto Mosqueda Taylor (2010). Oral medicine and pathology at a glance. Chichester, U.K.: Wiley-Blackwell. ISBN 978-1405199858.
  9. ↑ Woo, Sook-Bin (2012). Oral pathology : a comprehensive atlas and text (1st ed.). Philadelphia, PA: Elsevier/Saunders. pp. 154, 155. ISBN 978-1437722260.
  10. ↑ O'Neill, ID; Scully, C (October 2012). "Biologics in oral medicine: oral Crohn's disease and orofacial granulomatosis.". Oral diseases 18 (7): 633–8. doi:10.1111/j.1601-0825.2012.01918.x. PMID 22420719.
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