Digestive and Liver Disease 47 (2015) 338–341
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Orofacial granulomatosis in children: Think about Crohn’s disease Marzia Lazzerini a , Stefano Martelossi a , Gabriele Cont a , Chiara Bersanini b , Giovanna Ventura a,c , Massimo Fontana b , Giovanna Zuin b , Alessandro Ventura a,c , Andrea Taddio a,c,∗ a b c
Institute for Maternal and Child Health, IRCCS “Burlo Garofolo”, Trieste, Italy Department of Paediatrics, Ospedale Buzzi, Milan, Italy University of Trieste, Trieste, Italy
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Article history: Received 13 August 2014 Accepted 21 December 2014 Available online 30 December 2014 Keywords: Crohn’s disease Melkersson–Rosenthal syndrome Orofacial granulomatosis Thalidomide
a b s t r a c t Background: The term orofacial granulomatosis is conventionally used to describe patients with granulomatous lesions affecting the orofacial tissues, in absence of intestinal lesions. Lip swelling and facial swelling are the most common clinical signs. Despite the fact that histologically it is not distinguishable from Crohn’s disease, and that both diseases have a chronic/recurrent course, the relationship between orofacial granulomatosis and Crohn’s disease is still debated. Methods: Herein we present ﬁve cases of orofacial granulomatosis. Results: All patients presented concomitant Crohn’s disease, supporting the hypothesis that orofacial granulomatosis and Crohn’s disease may be one single disease. Thalidomide was effective in inducing remission of oral and intestinal symptoms in all ﬁve cases and could be considered a valid treatment opportunity for these patients. Conclusions: Orofacial granulomatosis and Crohn’s disease may be part of the same disease; both may respond to thalidomide. © 2014 Editrice Gastroenterologica Italiana S.r.l. Published by Elsevier Ltd. All rights reserved.
2. Materials and methods
Orofacial granulomatosis (OFG) consists in granulomatous lesions affecting orofacial tissues, in absence of intestinal lesions [1,2]. Lip swelling and facial swelling are the most common clinical signs of OFG [3,4]. Despite the fact that histologically OFG is not distinguishable from Crohn’s disease (CD) – both are characterised by non-caseating epithelioid cell granulomas – and that both diseases have a chronic/recurrent course, the relationship between OFG and CD is still being debated [1–4]. Currently it’s uncertain whether OFG is simply an oral presentation of CD, or a distinct inﬂammatory disorder [5,6].
Herein we present ﬁve cases of OFG which came to our attention between 2006 and 2012 at the IRCCS Burlo Garofolo of Trieste and Ospedale Buzzi of Milan, Italy.
∗ Corresponding author at: Institute for Maternal Child Health, IRCCS “Burlo Garofolo”, via dell’Istria 65/1, 34137 Trieste, Italy. Tel.: +39 040 3785 312; fax: +39 040 3785 290. E-mail address: [email protected]
3. Results 3.1. Case 1 A 7-year old boy presented a persistent gingival swelling and a recurrent swelling of both lips (Fig. 1A, B) with dry skin and diffuse hyperaemia over the cheeks and the forehead. He was treated with topical steroids, antibiotics, and antimycotic drugs without beneﬁt. No other symptoms were present. OFG diagnosis was made and he was treated with a course of oral prednisone 2 mg/kg/die, but unfortunately the disease relapsed soon after the patient was weaned off steroids. Two years after, based on the presence of perianal hyperaemia with small intergluteal ulcers, raised faecal calprotectin (545 mg/kg; n.v.