Orofacial Granulomatosis Research Articles

Sa1694 Capsule Endoscopy in Orofacial Granulomatosis Patients Commonly Diagnoses Crohn's Disease

Orofacial granulomatosis (OFG) is a chronic inflammatory disorder affecting the perioral tissues, and may be localised or have gastrointestinal involvement histologically identical to Crohn's disease (CD). We evaluated capsule endoscopy (CE) to screen for CD in patients presenting with OFG.

Orofacial granulomatosis: clinical study of 20 patients

The objective of this study was to analyze the clinical features of a series of patients with orofacial granulomatosis (OFG). Twenty patients diagnosed with OFG at Bellvitge Hospital (Barcelona, Spain) from 1985 to 2010 were included in the study. All of our patients (9 men and 11 women, median age 48.1 years) presented with labial swelling. Six patients presented with recurrent orofacial swelling, 12 with permanent swelling, and 2 with progressive swelling. Fissured tongue was observed in 9 cases, and 2 patients presented with recurrent episodes of peripheral facial paralysis. The median follow-up time was 65.1 months, ranging from 4 to 300 months. None of our patients developed sarcoidosis or Crohn disease. In the south of Europe, OFG does not appear to be as frequently associated with Crohn disease as in northern Europe. Although several treatments may achieve transient control of the orofacial swelling, there is no curative treatment for OFG and some patients may develop embarrassing lesions.

Response to Dr McNab

Sir, We thank Dr McNab1 for his comments on our report of isolated eyelid edema in Melkersson–Rosenthal Syndrome (MRS).2 He suggests that our cases are better considered examples of acne rosacea, and cites his own study3 and the case report of Lai et al4 in support of this. In Dr McNab's case series, histopathological examination of eyelid skin from five patients with a clinical diagnosis of acne rosacea and chronic eyelid edema showed some degree of granulomatous inflammation in three specimens.3 The granulomas were not illustrated but were described as ‘poorly formed' (case 4), ‘single' (case 11) and ‘surrounding dilated lymphatics' (case 15). Lai et al4 also referred to the presence of ‘ill-defined perivascular granulomas' but did not illustrate them. Other studies of chronic eyelid edema in rosacea did not mention dermal granulomas.5, 6 In none of our cases was rosacea felt to be the primary underlying cause, either clinically or histopathologically. In our practice, we do not regard poorly defined granulomas as indicative of any specific diagnosis. Granulomatous rosacea is typically characterised by a tuberculoid (necrotising) or sarcoid-like response, possibly to the contents of hair follicles. In our series, the granulomas were neither tuberculoid nor sarcoid-like but were sharply defined, perivascular, and perilymphatic, often with an intralymphatic component. In addition, and illustrated in our paper, discrete granulomas were identified in orbicularis muscle and anterior orbital soft tissue, which does not appear to have been described in rosacea. Dr McNab may be correct in saying that MRS is over diagnosed in cases of isolated eyelid edema.1 Nevertheless, the clinico-pathological pattern that we and other authors have ascribed to monosymptomatic MRS appears quite distinct. Until our understanding of the etiology and pathogenesis of oro-facial granulomatosis, of which MRS is one part, increases, we see no justification for regarding these cases as a form of granulomatous rosacea.

Biologics in oral medicine: oral Crohn’s disease and orofacial granulomatosis

Antitumour necrosis factor (TNF-α) therapy has a potential to benefit patients with oral lesions of Crohn's disease (CD) and patients with orofacial granulomatosis (OFG). The most appropriate use would appear to be in patients with severe or multisystem features, where other available agents have failed or have been associated with adverse effects. TNF-α antagonists (infliximab in particular) have a role in the management of orofacial CD and OFG, but potential adverse effects of TNF-α antagonists include acute infusion reactions, infection and increased risk of malignancy. Thus, a full risk-benefit analysis is indicated, with patient selection, use and subsequent monitoring coordinated with gastroenterologists with appropriate training and experience in biological therapies.

Orofacial granulomatosis presenting as bilateral eyelid swelling

Orofacial granulomatosis (OFG) is an uncommon but increasingly recognized disease of unknown etiology. The typical presentation is chronic swelling of the perioral soft tissue, but eyelid edema can be the sole manifestation. Terminology of this disease can be confusing as it may also be referred to as granulomatous cheilitis and a monosymptomatic presentation of Melkersson–Rosenthal syndrome (MRS). Crohn’s disease and sarcoidosis should also be considered in the differential as the histopathology can be similar. Corticosteroids are the mainstay of treatment but can lack efficacy. Atypical presentations and the possibility of systemic disease involvement can further challenge the management. We describe an unusual case in which OFG manifests solely as chronic eyelid swelling. This 69-year old Asian female patient had a delayed diagnosis that responded well to intralesional corticosteroid injection with surgical skin reduction. In addition to describing this unusual presentation of OFG, we review the relevant literature and evaluate the current terminology used to describe this entity.

Extraintestinal Crohn Disease Causing Bilateral Nasolacrimal Duct Obstruction

A 16-year-old boy presented with complaints of epiphora and bilateral palpably distended lacrimal sacs. He had a history of Crohn disease involving the small and large intestines, and a history of granulomatous cheilitis. Bilateral dacryocystorhinostomies were performed through an external approach and revealed multiple nodular masses in both lacrimal sacs, which on biopsy, contained granulomatous inflammation consistent with extraintestinal Crohn disease. A literature search from 1966 to the present using the PubMed database revealed three previous publications describing nasolacrimal duct obstruction associated with Crohn disease, but these cases did not show pathologic evidence of granulomatous inflammation. To our knowledge, this is the first reported case of extraintestinal Crohn disease with granulomatous inflammation affecting the lacrimal-outflow system.

Orofacial granulomatosis: A case report with review of literature

Orofacial granulomatosis (OFG) encompasses conditions characterized by non-necrotizing granulomatous inflammation of the oral and maxillofacial region that present clinically as labial enlargement, perioral and/or mucosal swelling, oral ulcerations, and gingivitis. The unifying term “OFG” has been introduced to integrate the spectrum of various disorders, including Melkersson-Rosenthal syndrome and granulomatous cheilitis (which is sometimes considered to be a monosymptomatic form of Melkersson-Rosenthal syndrome), and has been shown to be associated with Crohn's disease, sarcoidosis, and infectious diseases such as tuberculosis. Although various etiological agents such as food substances, food additives, dental materials, and various microbiological agents have been implicated in the disease process, its precise pathogenesis is yet to be elucidated. Delayed type of hypersensitivity reaction appears to play a significant role, although the exact antigen inducing the immunological reaction varies in individual patients. However, evidence for the role of genetic predisposition to the disease is sparse. The underlying immunological mechanism appears to show some similarities between OFG and Crohn's disease, emphasizing the need for more comparative studies of the two entities. The aim of this article is to report a case of OFG, along with a detailed literature review of the facts and variations associated with its nomenclature, clinical presentation, and etiology. It also projects the challenges that a professional has to face in the diagnosis and treatment planning of such cases.

Oral Crohn′s disease without intestinal manifestations

Crohn′s disease is a granulomatous inflammatory bowel disease and was described in 1932 as a chronic granulomatous disorder of the terminal ileum and is now considered a distinct member of the inflammatory bowel disease family. It may affect any part of the gastrointestinal tract. Oral Crohn′s disease has been reported frequently in the last three decades with or without intestinal manifestations. In the latter case, it is considered as one of the orofacial granulomatosis. There has been much doubt whether intestinal manifestations of Crohn′s disease will eventually develop in the orofacial granulomatosis. We present a female patient aged 22 years with prominent clinical findings such as persistent swelling of lower and upper lip with fissuring and angular cheilitis, granulomatous gingival enlargement, and cobblestone or corrugated appearance of labial mucosa, which are suggestive of Crohn′s disease, but with no evidence of other gastrointestinal involvement. The patient underwent surgical treatment with external gingivectomy procedure. A 6-month follow-up showed minimal recurrence.

Idiopathic orofacial granulomatosis in a young patient: A rare entity

The granular enlargement of the gingiva may be the first clinical manifestation of Orofacial granulomatosis, preceding other local or systemic manifestations. The term Idiopathic Orofacial granulomatosis (OFG) refers to conditions restricted to the oral region without any identifiable systemic granulomatous diseases. We report a case of nine year old female patient with gingival enlargement as the sole manifestation without any systemic involvement. Laboratory investigations were done to rule out tuberculosis, sarcoidosis, allergic hypersensitivity, Crohn's disease. The patient was treated by gingivectomy and there is complete remission of the lesion without any recurrence. Thus the early investigations and diagnosis of OFG can help in the prevention of future systemic complications.

A rare case report of orofacial granulomatosis in a pediatric patient

Orofacial granulomatosis comprises a group of diseases characterized by noncaseating granulomatous inflammation affecting the soft tissues of the oral and maxillofacial region. The most common clinical presentation is persistent swelling of one or both lips. It is important to establish the diagnosis accurately because this condition is sometimes a manifestation of Crohn's disease or sarcoidosis. This article describes a case of orofacial granulomatosis.

Orofacial Granulomatosis as First Manifestation of Crohn’s Disease: Successful Treatment of Both Conditions with a Combination of Infliximab and Dapsone

Orofacial Granulomatosis as First Manifestation of Crohn’s Disease: Successful Treatment of Both Conditions with a Combination of Infliximab and Dapsone

Successful treatment of granulomatous cheilitis with adalimumab

International Journal of DermatologyVolume 51, Issue 1 p. 118-120 Correspondence Successful treatment of granulomatous cheilitis with adalimumab Ricardo Ruiz Villaverde PhD, Ricardo Ruiz Villaverde PhD Dermatology Unit, Complejo Hospitalario de Jaen, SpainSearch for more papers by this authorDaniel Sánchez Cano MD, Daniel Sánchez Cano MD Dermatology Unit, Complejo Hospitalario de Jaen, SpainSearch for more papers by this author Ricardo Ruiz Villaverde PhD, Ricardo Ruiz Villaverde PhD Dermatology Unit, Complejo Hospitalario de Jaen, SpainSearch for more papers by this authorDaniel Sánchez Cano MD, Daniel Sánchez Cano MD Dermatology Unit, Complejo Hospitalario de Jaen, SpainSearch for more papers by this author First published: 20 December 2011 https://doi.org/10.1111/j.1365-4632.2010.04507.xCitations: 17 Ricardo Ruiz Villaverde, PhDEmir 15 Bloque 4 Portal 1 3° G 18006 Granada Spain E-mail: [email protected] Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinkedInRedditWechat Citing Literature Volume51, Issue1January 2012Pages 118-120 RelatedInformation

Further reliability and responsiveness of the Chronic Oral Mucosal Diseases Questionnaire

To test the reliability and responsiveness of the Chronic Oral Mucosal Diseases Questionnaire (COMDQ), in measuring the quality of life (QofL) in patients with chronic oral mucosal conditions. A random sample of 160 patients with the following chronic oral mucosal conditions, recurrent aphthous stomatitis, oral lichen planus, the more common vesiculobullous conditions (mucous membrane pemphigoid and pemphigus vulgaris) and orofacial granulomatosis received a copy of the COMDQ. A subset of 100 patients received the questionnaire on two further occasions, 2 weeks and 3 months later. Statistical tests were carried out to evaluate the test-retest reliability and responsiveness of this instrument. This study has demonstrated that the COMDQ has good test-retest reliability with an intraclass correlation coefficient of 0.81 and is responsive to changes in the patients' overall conditions. In conclusion, this study has further demonstrated the reliability and responsiveness of the COMDQ in assessing QofL in patients with chronic oral mucosal diseases.

Cheilitis granulomatosa

Melkersson-Rosenthal Syndrome (MRS) is a rare syndrome that is characterized by a triad of facial paralysis, chronic edema of the lip, and a fissured tongue. Most commonly, one element of the triad precedes the development of the other symptoms. We present a case of cheilitis granulomatosa (CG) as a manifestation of incomplete MRS. As the etiology remains unknown, treatment of CG is challenging. Intralesional glucocorticoids remain the first-line treatment, but recurrences are common. We discuss alternative treatment strategies that include combination therapy with other anti-inflammatory agents and biologics, such as infliximab.

Cheilitis granulomatosa treated with intralesional corticosteroids and anti-inflammatory agents

Cheilitis granulomatosa treated with intralesional corticosteroids and anti-inflammatory agents

Review article: cinnamon- and benzoate-free diet as a primary treatment for orofacial granulomatosis

Orofacial granulomatosis is a rare chronic granulomatous inflammatory disease of the lips, face and mouth. The aetiology remains unclear but may involve an allergic component. Improvements have been reported with cinnamon- and benzoate-free diets. To explore the prevalence of compound and food sensitivity and examine the dietary treatments used in orofacial granulomatosis. A comprehensive literature search was carried out and relevant studies from January 1933 to January 2010 were identified using the electronic database search engines; AGRIS 1991-2008, AMED 1985-2008, British Nursing and Index archive 1985-2008, EMBASE 1980-2008, evidence based medicine review databases (e.g. Cochrane DSR), International Pharmaceutical and Medline 1950-2008. Common sensitivities identified, predominantly through patch testing, were to benzoic acid (36%) food additives (33%), perfumes and flavourings (28%), cinnamaldehyde (27%), cinnamon (17%), benzoates (17%) and chocolate (11%). The cinnamon- and benzoate-free diet has been shown to provide benefit in 54-78% of patients with 23% requiring no adjunctive therapies. A negative or positive patch test result to cinnamaldehyde, and benzoates did not predict dietary outcome. The most concentrated source of benzoate exposure is from food preservatives. Use of liquid enteral formulas can offer a further dietary therapy, particularly in children with orofacial granulomatosis. Management of orofacial granulomatosis is challenging but cinnamon- and benzoate-free diets appear to have a definite role to play.

Intralymphatic Granulomas as a Pathogenic Factor in Cheilitis Granulomatosa/Melkersson–Rosenthal Syndrome: Report of a Case With Immunohistochemical and Molecular Studies

Orofacial granulomatosis, an uncommon immunologically mediated disorder, includes cheilitis granulomatosa and Melkersson-Rosenthal syndrome. It is clinically characterized by recurrent or persistent swelling of the orofacial tissues with a spectrum of other orofacial features and sometimes with neurological symptoms. The pathological findings are varied but are often characterized by the presence of noncaseating granuloma. We present a new case of orofacial granulomatosis with unusual histopathological findings, namely, intralymphatic granulomas. These may be the cause of the tissue edema. We demonstrated, by immunohistochemical studies, the lymphatic nature of the vessels affected by the granulomatous process.

Characteristics of patients with orofacial granulomatosis

Orofacial granulomatosis has mostly been described in reports of very small numbers of cases. Few large case groups have been described. The aim of this study was to describe the demographics, symptoms, clinical features and laboratory findings in a large cohort of cases. Clinical and laboratory data for 119 cases of orofacial granulomatosis who attended oral medicine clinics in Dublin, Ireland, were examined for demographic characteristics at the time of first presentation. The male/female ratio was approximately 1:1, with a median age (and range) of 28 (5-84) years. Symptoms had been present for a median duration of 12 weeks. A food association was suspected by 30% of patients. The predominant complaint was lip swelling (77%) with only 15% reporting facial swelling, while 8% complained of both. Almost all patients had clinical evidence of lip or facial swelling (95%). Other common extra-oral manifestations were lip fissuring (30%), angular cheilitis (28%) and perioral erythema (28%). Common intra-oral manifestations were cobblestoning of the buccal mucosa (63%), ulcers (36%), granulomatous gingivitis (33%), mucosal tags (29%) and fissured tongue (17%). Over half of the biopsies (56%) performed were reported as typical of orofacial granulomatosis. This is one of the largest cohorts of orofacial granulomatosis patients to have been described in detail.

Asymptomatic Granulomatous Vulvitis and Granulomatous Cheilitis in Childhood: The Need for Crohn Disease Workup

Asymptomatic Granulomatous Vulvitis and Granulomatous Cheilitis in Childhood: The Need for Crohn Disease Workup

Orofacial granulomatosis associated with hypersensitivity to dental amalgam

Orofacial granulomatosis is a clinicopathologic entity characterized by chronic swelling of the lip and possible soft tissues in the orofacial region owing to granulomatous inflammation of unknown cause. We present 3 cases of orofacial granulomatosis associated with allergic contact dermatitis to dental materials. Previous treatment with corticosteroids did not have any therapeutic effect. Patch testing revealed a positive reaction to several allergens, including dental amalgam and mercury in 2 cases. A lymphocyte transformation test modified for metals was used to evaluate lymphocyte reactivity. After the removal of the suspected allergens, all patients experienced recovery within 1 month, with the exception of the lip swelling, which gradually subsided over several months.