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References

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Lazzerini M, Bramuzzo M, Ventura A. Association between orofacial granulomatosis and Crohn's disease in children: systematic review. World J Gastroenterol. 2014; 20:7497-7504
Al-Hamad A, Porter S, Fedele S. Orofacial granulomatosis. Dermatol Clin. 2015; 33:433-446
Campbell H, Escudier M, Patel P Distinguishing orofacial granulomatosis from crohn's disease: two separate disease entities?. Inflamm Bowel Dis. 2011; 17:2109-2115
Al Johani KA, Moles DR, Hodgson TA, Porter SR, Fedele S. Orofacial granulomatosis: clinical features and long-term outcome of therapy. J Am Acad Dermatol. 2010; 62:611-620
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Tilakaratne WM, Freysdottir J, Fortune F. Orofacial granulomatosis: review on aetiology and pathogenesis. J Oral Pathol Med. 2008; 37:191-195
Gibson J, Wray D. Human leucocyte antigen typing in orofacial granulomatosis. Br J Dermatol. 2000; 143:1119-1121
Freysdottir J, Zhang S, Tilakaratne WM, Fortune F. Oral biopsies from patients with orofacial granulomatosis with histology resembling Crohn's disease have a prominent Th1 environment. Inflamm Bowel Dis. 2007; 13:439-445
Fitzpatrick L, Healy CM, McCartan BE, Flint SR, McCreary CE, Rogers S. Patch testing for food-associated allergies in orofacial granulomatosis. J Oral Pathol Med. 2011; 40:10-13
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Orofacial granulomatosis in children − a review

From Volume 46, Issue 1, January 2019 | Pages 42-48

Authors

Maalini Patel

BDS(Hons), MPaedDent RCSEng, FDS RCSEng

Specialty Registrar in Paediatric Dentistry, Guy's and St Thomas' NHS Foundation Trust, Bristol, UK

Articles by Maalini Patel

Gemma Davis

BDS, MFDSRCS (Eng), PgCertTLHP

Specialty Registrar in Oral Medicine, University Hospitals Bristol NHS Foundation Trust, Bristol, UK

Articles by Gemma Davis

Rebecca John

BDS, MPaed Dent RCS(Ed), FDS RCS(Ed)

Consultant Senior Lecturer in Paediatric Dentistry, University Hospitals Bristol NHS Foundation Trust, Bristol, UK

Articles by Rebecca John

Rachel Cowie

BDS, MBChB MFDS RCS(Ed) FDS(OM) RCS(Ed)

Consultant in Oral Medicine, University Hospitals Bristol NHS Foundation Trust, Bristol, UK

Articles by Rachel Cowie

Abstract

Abstract: Orofacial granulomatosis (OFG) is an uncommon chronic inflammatory disorder that can present in childhood. It has a range of clinical manifestations with the common features being lip swelling and oral ulceration. It can be idiopathic or associated with systemic granulomatous conditions such as Crohn's disease and sarcoidosis. Patients presenting with features suggestive of OFG, with or without gastrointestinal symptoms, should be referred promptly to secondary care. The management of OFG is often challenging and includes dietary restrictions; topical, intralesional and systemic corticosteroids; and other systemic immunomodulatory drugs. This condition can lead to significant psychological morbidity for the child if left untreated.

CPD/Clinical Relevance: Orofacial granulomatosis can present in childhood and its features may be first noted on routine check-up with a general dental practitioner (GDP).

Article

Orofacial granulomatosis (OFG) is a rare condition affecting both children and adults. It is characterized by granulomatous lesions affecting the orofacial region. It can appear as an isolated condition, the true definition of OFG.1 It is a diagnosis of exclusion as its features can present in conjunction with systemic granulomatous disorders such a Crohn's Disease (CD) and sarcoidosis.2 There has been speculation that OFG may be a predictor of future CD when seen in children.3 This article will focus on the paediatric patient presenting with clinical features of orofacial granulomatous lesions, including those later found to have CD.

Orofacial granulomatosis was first described in 1985 by Weisenfeld et al, who reported 60 cases presenting with lymphoedema and the presence of multiple non-caseating giant cell granulomatous lesions.4 It is closely related to Melkersson-Rosenthal syndrome which describes the triad of persistent lip or facial swelling, recurrent facial paralysis and fissured tongue.1

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