Treatment Of Orofacial Granulomatosis Research Articles

Опыт лечения орофациального гранулематоза, представленного макрохейлитом Мишера, циклоспорином А

Опыт лечения орофациального гранулематоза, представленного макрохейлитом Мишера, циклоспорином А

OROFACIAL GRANULOMATOSIS – A REVIEW

Orofacial Granulomatosis (OFG) is a rare clinicopathological entity characterized clinically by the presence of continuous enlargement of the soft tissues of the oral and maxillofacial region and histologically by non-caseating and non-necrotizing granulomatous inammation. The term 'orofacial granulomatosis' has been introduced to indicate the group of various disorders, involving Melkersson-Rosenthal syndrome and granulomatous cheilitis and has been noted to be associated with Sarcoidosis, Crohn's disease and infectious diseases such as Tuberculosis. But, various etiological agents such as food additives, dental materials and microbial agents have been recommended in the disease process. Treatment of orofacial granulomatosis is by corticosteroids but it's not so efcient. It is more important to nd the pathogen rst to specify the appropriate treatment line.

Management of orofacial granulomatosis

Orofacial granulomatosis is a chronic relapsing-remitting inflammatory condition that shares a similar phenotypic presentation to some other granulomatous diseases, particularly Crohn's disease. However, subtle clinical and pathological differences justify it as a separate disease entity. Previous studies have assessed the effectiveness of interventions used in the management of orofacial granulomatosis. This article reviews the management options available. A literature search was conducted to identify studies, in English, which assessed the effect of non-pharmacological and pharmacological interventions in the treatment of orofacial granulomatosis. The interventions were categorised into dietary modification, pharmacological (topical, intralesional and systemic therapy), surgery and psychological. A combination of interventions is often required to effectively manage each patient. There is convincing evidence that diet plays a role in disease severity. In patients where dietary manipulation alone is unsuccessful, topical, intralesional and/or systemic treatment may be considered to manage the condition.

Interdisciplinary approach in the diagnosis and treatment of orofacial granulomatosis

Orofacial granulomatosis (OFG) is an uncommon disease and represents an idiopathic, nonnecrotizing granulomatous lesion of soft tissue in the orofacial region. Medical history is very important in the diagnosis of OFG as medical condition such as Crohn's disease can also present an extraintestinal manifestation of OFG. Exclusion of other entities such as sarcoidosis, myocardial infections, foreign body reactions, and biopsy helps establish a correct diagnosis. The term idiopathic OFG is used in cases with unknown etiology. This case report describes idiopathic OFG in a 35-year-old female patient with persistent upper lip swelling and gingival enlargement. Management with intralesional acetonide 20 mg/ml, twice week for 3 weeks and gingivectomy, resulted in significant remission without recurrence after a 6-month follow-up.

Orofacial Granulomatosis: A Review

Orofacial Granulomatosis (OFG) is an uncommon clinicopathological entity characterized clinically by the presence of persistent enlargement of the soft tissues of the oral and maxillofacial region and histologically by non-caseating and non-necrotizing granulomatous inflammation. The term ‘orofacial granulomatosis’ has been introduced to denote the group of various disorders, including Melkersson-Rosenthal syndrome and granulomatous cheilitis and has been noted to be associated with Crohn's disease, Sarcoidosis and infectious diseases such as Tuberculosis. Although, various etiological agents such as dental materials, food additives and microbial agents have been recommended in the disease process. Treatment of orofacial granulomatosis is by corticosteroids but it's not so efficient. It is more important to identify the pathogen first to specify the appropriate treatment line.

Treatment of orofacial granulomatosis: a case report

BackgroundOrofacial granulomatosis is a relatively recent term coined by Wiesenfield et al. in 1985 to define granulomatous lesions of oral mucosa without intestinal involvement. When it presents in a triad encompassing facial nerve palsy, lip swelling, and fissured or furrowed tongue it is called Melkersson–Rosenthal syndrome while monosymptomatic or oligosymptomatic forms are referred to as granulomatous cheilitis. It is an uncommon clinicopathologic entity which is distinct from classic Crohn’s disease. The NOD2 variant which is commonly associated with Crohn’s has not been shown to have any association with orofacial granulomatosis.Case presentationWe present a case of a 31-year-old white man who had painful swelling of the lip with oral ulcers and difficulty eating for 2 to 3 years. He was diagnosed as having granulomatous cheilitis based on characteristic biopsy findings. There was serologic evidence of Crohn’s disease with anti-Saccharomyces cerevisiae antibodies. However, he was not found to have any gastrointestinal involvement based on computed tomography enterography, and upper and lower endoscopies. He failed to respond to nonsteroidal anti-inflammatory drugs, steroids, and dapsone therapy but responded well to high doses of infliximab.ConclusionsOur case questions whether granulomatous cheilitis really exists or is it simply a variant of Crohn’s disease with only oral presentation. Our patient did not have symptoms of Crohn’s disease; moreover, endoscopic studies and computed tomography enterography were unremarkable for evidence of intestinal involvement. Our case is also the first reported case where high-dose infliximab alone has been used with sustained response for approximately 8 months. In conclusion, more research is needed to assess the underlying pathology as well as ideal treatment options for patients with orofacial granulomatosis. We propose that high-dose infliximab should be considered in patients who do not respond to traditional therapies.

Azathioprine is effective for oral involvement in Crohn's disease but not for orofacial granulomatosis alone.

There have been no previous reports assessing the effectiveness of azathioprine (AZA) in the treatment of orofacial granulomatosis (OFG). This report is a review of patients receiving AZA for active OFG with or without concomitant gut Crohn's disease (CD) in a specialist tertiary referral centre. Clinical response was defined by Global Physician Assessment at 4-, 12- and 24-month follow-up and a standardised oral disease activity score (ODAS). Sixty of 215 patients seen with OFG in our clinic over a 12-year period were treated with AZA. Of these, 22 had concomitant CD. The proportion of patients responding to AZA with a diagnosis of CD/OFG vs. OFG only at 4, 12 and 24 months were 54% vs. 21% (P = 0.03), 59% vs. 21% (P = 0.003) and 41% vs. 24% (P = 0.16), respectively. A statistically significant difference was seen between starting and follow-up ODAS scores at 4 months in the CD/OFG group which was not observed in the OFG only group. Factors predicting a need for AZA included a diagnosis of intestinal CD, sulcal swelling, sulcal ulcers and upper lip involvement. The factor predicting response to treatment was a diagnosis of CD at 12 months of follow-up. No difference in the number of adverse effects was observed between the two groups of patients. AZA is significantly more effective in the treatment of oral disease with a concurrent diagnosis of CD rather than in the treatment of OFG alone.

Orofacial Granulomatosis.

Orofacial granulomatosis (OFG) is an uncommon chronic inflammatory disorder of the orofacial region. It is characterized by subepithelial noncaseating granulomas and has a spectrum of possible clinical manifestations ranging from subtle oral mucosal swelling to permanent disfiguring fibrous swelling of the lips and face. Etiopathogenesis is unknown. A range of systemic granulomatous disorders, including Crohn disease and sarcoidosis, may cause orofacial manifestations that cannot be distinguished from those of OFG. Treatment of OFG has proven difficult and unsatisfactory, with no single therapeutic model showing consistent efficacy in reducing orofacial swelling and mucosal inflammation.

A rare case of primary orofacial granulomatosis of gingiva during pregnancy

Orofacial granulomatosis (OFG) comprises a group of diseases characterized by noncaseating granulomatous inflammation affecting the soft tissues of the oral and maxillofacial region. It is important to establish the diagnosis accurately because this condition is sometimes a manifestation of many systemic conditions like Crohn's disease or sarcoidosis. The clinical outcome of OFG patients continues to be unpredictable. Current therapies remain unsatisfactory. This article reports a rare case of isolated OFG with langhans type giant cells and inflammatory infiltrate without any systemic involvement, in which the condition was a manifestation of pregnancy. The diagnostic approach to and the treatment of OFG are reviewed.

Clinical behaviour and long‐term therapeutic response in orofacial granulomatosis patients treated with intralesional triamcinolone acetonide injections alone or in combination with topical pimecrolimus 1%

Orofacial granulomatosis (OFG) is a relapsing inflammatory disorder of unknown aetiology and non-standardized treatment protocols. The aim of this study was to assess the clinical behaviour and long-term therapeutic response in OFG patients treated with intralesional triamcinolone acetonide (TA) injections alone or in combination with topical pimecrolimus 1%, as adjuvant, in those patients partially responders to TA. We analysed data from 19 OFG patients followed-up for 7 years. Demographic characteristics, clinical behaviour and long-term therapeutic response were investigated. Eleven (57.9%) OFG patients treated with intralesional TA injections therapy reached first complete clinical remission in a mean time of 10 ± 2.2 (95% CI, 8.5-11.5) weeks, while eight (42.1%) patients, partially responders to intralesional TA injections, were treated with TA injections plus topical pimecrolimus 1%, as adjuvant, achieving complete clinical remission in a mean time of 29.8 ± 7.8 (95% CI, 23.2-36.3) weeks. Relapses occurred in four TA responder patients with a disease-free time of 35.8 ± 8.7 (95% CI, 21.9-46.4) weeks and in five patients treated with TA and topical pimecrolimus 1% with a disease-free time of 55.8 ± 18.5 (95% CI, 32.8-78.8) weeks. Patients were followed-up for a mean time of 56.3 ± 18.2 (95% CI, 47.6-65.1) months. At last control, all 19 patients were in complete clinical remission. These preliminary data suggest that intralesional TA injections still represent a mainstay in the treatment of OFG. It is unclear the role of topical pimecrolimus, as adjuvant, in leading OFG patients, partly responders to intralesional TA injections, to a complete clinical remission.

Review article: orofacial granulomatosis

Orofacial granulomatosis is an uncommon clinicopathological entity describing patients who have oral lesions characterized by persistent and/or recurrent labial enlargement, oral ulcers and a variety of other orofacial features, who on lesional biopsy have lymphoedema and non-caseating granulomas. The aetiology of oral lesions with non-caseating granulomas includes oral Crohn's disease (some patients with oral lesions will develop typical bowel symptoms of Crohn's disease in ensuing months to years), tooth-associated infections, sarcoidosis and food or contact allergies. Treatment of orofacial granulomatosis is not reliably effective and may not be always necessary, although most patients do require some medical intervention.

Intralesional Steroid Injection After Nerve Block Anesthesia in the Treatment of Orofacial Granulomatosis

The facial disfigurement produced by orofacial granulomatosis causes enormous embarrassment. None of the many therapies recommended is reliably successful. Oral corticosteroids cause significant side effects and repeated injections of small quantities of triamcinolone are painful. We have injected large volumes of triamcinolone after numbing the lips using nerve block anesthesia. Nine patients (six males and three females, aged 10 to 47 years) with orofacial granulomatosis were investigated. No evidence of an allergic cause was found using patch or contact urticaria tests. Eating chocolate produced lip swelling in one man, and his lip shrank in size after avoiding this for 12 months. Five patients, aged 10 to 24 years, were treated with high-volume intralesional triamcinolone injections (3 to 10 mL of 10 mg/mL) after first numbing the lips using infraorbital nerve branch and mental nerve block. After 6 weeks, the lip size returned to normal in four patients and was reduced in a fifth. One patient was injected on four occasions over a 2-year period; in four other patients treated once, lip size remained reduced for over 10 months. Intralesional triamcinolone reduces lip swelling in patients with orofacial granulomatosis. Numbing the lips by nerve block anesthesia before triamcinolone injection enables adequate volumes and repeated injections to be given painlessly.

Intralesional steroid injection after nerve block anesthesia in the treatment of orofacial granulomatosis

Background.-The facial disfigurement produced by orofacial granulomatosis causes enormous embarrassment. None of the many therapies recommended is reliably successful. Oral corticosteroids cause significant side effects and repeated injections of small quantities of triamcinolone are painful. We have injected large volumes of triamcinolone after numbing the lips using nerve block anesthesia. Observations.-Nine patients (six males and three females, aged 10 to 47 years) with orofacial granulomatosis were investigated. No evidence of an allergic cause was found using patch or contact urticaria tests. Eating chocolate produced lip swelling in one man, and his lip shrank in size after avoiding this for 12 months