Gingival Orofacial Granulomatosis Clinical and 2D/3D Microscopy Features after Orthodontic Therapy: A Pediatric Case Report.

Orofacial granulomatosis (OFG) represents a heterogeneous group of rare orofacial diseases. When affecting gingiva, it appears as a chronic soft tissue inflammation, sometimes combined with the enlargement and swelling of other intraoral sites, including the lips. Gingival biopsy highlights noncaseating granulomatous inflammation, similar to that observed in Crohn's disease and sarcoidosis. At present, the etiology of OFG remains uncertain, although the involvement of the genetic background and environmental triggers, such as oral conditions or therapies (including orthodontic treatment), has been suggested. The present study reports the results of a detailed clinical and 2D/3D microscopy investigation of a case of gingival orofacial granulomatosis in an 8-year-old male patient after orthodontic therapy. Intraoral examination showed an erythematous hyperplasia of the whole gingiva with a granular appearance occurring a few weeks after the installation of a quad-helix. Peri-oral inspection revealed upper labial swelling and angular cheilitis. General investigations did not report ongoing extra-oral disturbances with the exception of a weakly positive anti-Saccharomyces cerevicae IgG auto-antibody. Two- and three-dimensional microscopic investigations confirmed the presence of gingival orofacial granulomatosis. Daily corticoid mouthwashes over a period of 3 months resulted in a slight improvement in clinical signs, despite an intermittent inflammation recurrence. This study brings new insights into the microscopic features of gingival orofacial granulomatosis, thus providing key elements to oral practitioners to ensure accurate and timely OFG diagnosis. The accurate diagnosis of OFG allows targeted management of symptoms and patient monitoring over time, along with early detection and treatment of extra-oral manifestations, such as Crohn's disease.

Orofacial granulomatosis associated with gingival enlargement: a case report.

Orofacial granulomatosis (OFG) is an uncommon condition with varying clinical presentation. Gingival enlargement in children could be due to a varied etiology. The present case report is of an adolescent female with initial presentation of generalized gingival enlargement, lip swelling and perioral discoloration without any known etiopathological factors or systemic involvement. Conservative excision of the enlargement was performed and histopathological examination revealed a non caseating granulomatous lesion. Diagnosis of orofacial granulomatosis in context to sarcoidosis was arrived after excluding other granulomatous diseases. Follow up after 18 months showed no recurrence and regression of lip swelling and perioral discoloration. Gingival enlargement can be considered as one of the presenting features of sarcoidosis.

Pediatric patients with orofacial granulomatosis likely to subsequently develop intestinal Crohn's disease: Brief Report.

Orofacial granulomatosis (OFG) is an uncommon chronic granulomatous condition presenting as perioral inflammation in the absence of systemic disease. There is continued debate regarding whether OFG is a distinct clinical disorder or a manifestation of orofacial Crohn's disease. Our retrospective review identified 7 patients diagnosed with OFG between 2000 and 2018 at a tertiary pediatric hospital. Four of the 7 patients subsequently developed Crohn's disease with a median delay of 3.1 years (range 0.4-6.9 years). This indicates that gastroenterology evaluation with long-term monitoring for intestinal Crohn's disease is warranted.

Detailed Follow-up Study of Pediatric Orofacial Granulomatosis Patients.

OBJECTIVES: Orofacial granulomatosis (OFG) is a chronic inflammatory condition affecting the orofacial area. Its connection to Crohn disease (CD) is debated. Our aim was to describe a cohort of pediatric patients with OFG in detail, study the long-term behavior of OFG, and evaluate factors predicting CD in patients with OFG. METHODS: We invited patients diagnosed with OFG at 2 university hospitals, Finland for a follow-up appointment. Patients (n = 29) were examined by a dentist and an otorhinolaryngologist using a structural schema. Orofacial findings were also recorded using digital photographing. Patients filled in questionnaires about general health and special diets. Patients' nutrition was evaluated from food records. The findings were compared between patients with OFG only and OFG with CD. RESULTS: Patients with CD had more findings in the orofacial area (total score for orofacial findings median 11) compared to patients with OFG only (total score median 7.5). There was no statistically significant difference in the type of lesions between these groups, except the upper lip was more often affected in patients with CD (n = 11) than in patients with OFG only (n = 0). Most of the patients had normal otorhinolaryngological findings. All patients with elevated anti-Saccharomyces cerevisiae antibody A levels had CD (n = 6) and they presented with more orofacial findings (total score) than patients with normal levels of anti-S cerevisiae antibody A (P = 0.0311). CONCLUSIONS: Long-term follow-up of pediatric-onset patients with OFG shows good prognosis. Patients with OFG do not seem to have otorhinolaryngological comorbidity. Anti-S cerevisiae antibody A may serve as a factor to indicate the possible presence of underlying CD in patients with OFG, but further studies are requested.

Adverse reaction to silicone simulating orofacial granulomatosis.

BACKGROUND: Granulomatous reactions to silicone facial fillers are well described in the literature. Clinically, these reactions present as nodules or pseudotumors that are frequently described as silicone granulomas or siliconomas. OBJECTIVE: We want to report a peculiar form of granulomatous reaction to injected silicone characterized by recurrent episodes of facial edema. METHODS: We collected silicone infiltrated patients with a similar clinical picture consisting of asymptomatic episodes of unilateral facial edema that had been recurring for months or years. RESULTS: We found four women with recurrent episodes of facial edema. They had been infiltrated with silicone in the face. Histology showed silicone deposits and a granulomatous infiltrate in all 4 cases. CONCLUSION: We describe and illustrate a new type of adverse reaction to injected silicone simulating orofacial granulomatosis. The reaction presents as recurrent, unilateral, asymmetric facial edema of the cheek in patients who have been injected with silicone in the face. Familiarity with this adverse reaction will help to prevent erroneous diagnoses such as idiopathic angioedema, Melkersson Rosenthal syndrome, and orofacial granulomatosis.

Orofacial granulomatosis: clinical signs of different pathologies.

Orofacial granulomatosis (OFG) is an uncommon disease characterized by persistent or recurrent soft tissue enlargement, oral ulceration and a variety of other orofacial features. It could be an oral manifestation of a systemic disease. For a correct differential diagnosis, local and systemic conditions characterized by granulomatous inflammation should be excluded using appropriate clinical and laboratory investigations. In fact, the diagnosis of OFG may be confirmed only by histopathological identification of noncaseating granulomas. The literature from 1943 to 2014 was reviewed with emphasis on the etiology of OFG and on clinical manifestations of systemic pathologies associated with OFG. The precise cause of OFG is still unknown, although several theories have been suggested, such as infection, hereditary factors and allergy. OFG is a disease that has a wide spectrum of presentation, which may include the oral manifestation of a systemic condition such as Crohn's disease, sarcoidosis, granulomatosis with polyangiitis and Melkersson-Rosenthal syndrome.

Unusual manifestations of a common gastrointestinal disorder.

We present an instructive case of a 13-year old male who presented with bilateral scrotal redness, swelling and tenderness, but with a normal testicular exam. His scrotal swelling persisted despite treatment with intravenous antibiotics, and on further history he reported 2 years of intermittent upper lip swelling. After a referral to a dermatologist, a lip biopsy showed granulomatous changes and he was referred to the gastroenterology department. A gastroscopy and colonoscopy was performed and histology confirmed non-caseating granulomas consistent with Crohn's disease (CD). Eighteen months after the diagnosis of CD he developed perianal disease with a fistula and distal anal stricture. He was successfully treated with insertion of a seton and escalation of therapy to azathioprine and infliximab. CD is a phenotypically diverse chronic inflammatory condition with an increasing incidence in Australia and other Western countries. Non-typical presentations, such as perianal manifestations or orofacial granulomatosis, can be the only presenting symptom in CD, and this highlights the importance for a high degree of clinical suspicion. Genital involvement is rare, but reported.

Causes of Oral Granulomatous Disorders: An Update and Narrative Review of the Literature.

Granulomatous diseases include a diverse range of chronic inflammatory disorders with a wide variety of pathologies and clinical characteristics. In particular, the orofacial region can be affected by granulomatous conditions-whether as an isolated disease or as part of a systemic disorder. Regardless of the nature of the disease or its mechanism of development, precise diagnosis can be challenging, as etiopathogenesis may be driven by several causes. These include reactions to foreign bodies, infections, immune dysregulation, proliferative disorders,, medications, illicit drugs, and hereditary disorders. Granulomas can be identified using histopathological assessment but are not pathognomonic of a specific disease, and therefore require correlation between clinical, serological, radiographical, and histopathological findings. The purpose of this review is to provide a summary of the etiopathogenesis, clinical and histopathologic characteristics, and treatment of oral granulomatous disorders.

Orofacial granulomatosis related to amalgam fillings.

This paper reports on a case of Orofacial Granulomatosis (OFG) in which the presence of amalgam fillings appears to have played a part in the aetiology. Once these restorations were removed and replaced with an alternative composite restorative material, all symptoms and signs of OFG resolved completely. This case highlights the necessity to include dental metals in the patch test battery when performing delayed patch testing on patients with OFG.

Identification of Putative Bacterial Pathogens for Orofacial Granulomatosis Based on 16S rRNA Metagenomic Analysis.

Orofacial granulomatosis (OFG) is a chronic inflammatory disease characterized by nontender swelling of the orofacial tissues, the underlying cause of which remains unknown. Our previous study demonstrated that tooth apical periodontitis (AP) is involved in the development of OFG. To characterize the AP bacterial signatures of OFG patients and identify possible pathogenic bacteria that cause OFG, the compositions of the AP microbiotas in OFG patients and controls were compared using 16S rRNA gene sequencing. Pure cultures of putative bacterial pathogens were established by growing bacteria as colonies followed by purification, identification, and enrichment and then were injected into animal models to determine the causative bacteria contributing to OFG. A specific AP microbiota signature in the OFG patients was shown, characterized by the predominance of phyla Firmicutes and Proteobacteria, notably members of the genera Streptococcus, Lactobacillus, and Neisseria, were found. Streptococcus spp., Lactobacillus casei, Neisseria subflava, Veillonella parvula, and Actinomyces spp. from OFG patients were isolated and successfully cultured in vitro and then injected into mice. Ultimately, footpad injection with N. subflava elicited granulomatous inflammation. IMPORTANCE Infectious agents have long been considered to play a role in the initiation of OFG; however, a direct causal relationship between microbes and OFG has not yet been established. In this study, a unique AP microbiota signature was identified in OFG patients. Moreover, we successfully isolated candidate bacteria from AP lesions of OFG patients and assessed their pathogenicity in laboratory mice. Findings from this study may help provide in-depth insights into the role of microbes in OFG development, providing the basis for targeted therapeutic approaches for OFG.

Tic douloureux as a presenting feature of facial leprosy: diagnostic enigma in Taiwan.

PURPOSE: Leprosy is rarely seen in Taiwan. We herein report a foreign worker concomitantly with facial borderline tuberculoid leprosy presenting with trigeminal neuralgia. CASE REPORT: A 26-year-old male foreign labor from Indonesia, presented with 1 year history of a hypoanaesthetic erythematous plaque of right face and subsequent 6 months constant, severe pain in the right side of his face over the nasolabial groove. Biopsies and histopathological examination confirmed the diagnosis of leprosy. We treated the patient with a multidrug regimen including dapsone, clofazimine, and rifampine since April of 2012 with a good response. CONCLUSIONS: We report a rare case of new-onset leprosy presenting with trigeminal neuralgia in Taiwan and suggest leprosy should be listed in the differential diagnosis of unusual skin manifestations and neuralgia.

Orofacial Granulomatosis Associated with Crohn's Disease: a Multicentre Case Series.

BACKGROUND: Orofacial granulomatosis [OFG] is a rare syndrome that may be associated with Crohn's disease [CD]. We aimed to characterise this relationship and the management options in the biologic era. METHODS: This multicentre case series was supported by the European Crohn's and Colitis Organisation [ECCO], and performed as part of the Collaborative Network of Exceptionally Rare case reports [CONFER] project. Clinical data were recorded in a standardised collection form. RESULTS: This report includes 28 patients with OFG associated with CD: 14 males (mean age of 32 years, ±12.4 standard deviation [SD]) and 14 females [40.3 years, ±21.0 SD]. Non-oral upper gastrointestinal tract involvement was seen in six cases and perianal disease in 11. The diagnosis of OFG was made before CD diagnosis in two patients, concurrently in eight, and after CD diagnosis in 18. The distribution of OFG involved the lips in 16 cases and buccal mucosa in 18. Pain was present in 25 cases, with impaired swallowing or speaking in six. Remission was achieved in 23 patients, notably with the use of anti-tumour necrosis factors [TNFs] in nine patients, vedolizumab in one, ustekinumab in one, and thalidomide in two. A further five cases were resistant to therapies including anti-TNFs. CONCLUSIONS: OFG associated with CD may occur before, concurrently with, or after the diagnosis of CD. Perianal and upper gastrointestinal [UGI] disease are common associations and there is a significant symptom burden in many. Remission can be obtained with a variety of immunosuppressive treatments, including several biologics approved for CD.

Orofacial granulomatosis--a 20-year review.

Orofacial granulomatosis (OFG) is the presence of persistent enlargement of the soft tissues of the oral and maxillofacial region, characterized by non-caseating granulomatous inflammation in the absence of diagnosable systemic Crohn's disease (CD) or sarcoidosis. Over 20 years have passed since OFG was first described and an extensive review of the literature reveals that there is no consensus whether OFG is a distinct clinical disorder or an initial presentation of CD or sarcoidosis. Furthermore, the precise cause of OFG is still unknown although several theories have been suggested including infection, genetic predisposition and allergy. The clinical outcome of OFG patients continues to be unpredictable. Current therapies remain unsatisfactory. Regular clinical review is indicated to identify the development of gastrointestinal or systemic involvement. The aim of this review was to analyse the developments in our understanding of the aetiology, pathogenesis and treatment protocols, with particular emphasis on management and outcomes of OFG since this entity was first described in 1985.

Non-infectious Granulomatous Lesions of the Orofacial Region.

Granulomatous lesions of the orofacial region are a heterogeneous group of disorders characterized by a granulomatous reaction to a variety of stimuli. Infectious agents, foreign material, systemic inflammation and metabolic disorders can all be associated with granulomatous inflammation. In the orofacial region primary causes of granulomatosis include foreign body reaction, delayed hypersensitivity to topical agents and idiopathic orofacial granulomatosis. Secondary causes of granulomas include infectious agents, sarcoid, and Crohn disease. For this review, infectious causes of orofacial granulomatosis (OFG) including bacteria, parasites and fungi will not be discussed.

Orofacial granulomatosis: review on aetiology and pathogenesis.

Orofacial granulomatosis (OFG) is considered as an uncommon disease and nomenclature of the disease was subjected to debate for a long time. Although various aetiological 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. Therefore, we propose the term idiopathic OFG as a better term for those cases restricted to oral region without any identifiable known granulomatous disease and the diagnosis should not be changed until the patient develops systemic manifestations of a specific granulomatous condition. This review attempts to discuss the role of different aetiological agents and certain aspects of pathogenesis of OFG.

Diagnosis and Treatment of Orofacial Granulomatosis.

Orofacial granulomatosis (OFG) is a chronic inflammatory disorder characterized by lip swelling and gingival alterations. OFG occurs either as a separate clinical entity or associated with a systemic disorder such as Crohn's disease (CD). The purpose of this paper is to report the case of a 13-year old boy who presented with marked granulomatous gingival inflammation and stomach discomfort. Periodontal therapy resulted in only slight reduction in gingival inflammation and enlargement. The diagnosis of OFG, which was evidently associated with inflammation in the bowel, was based on the gingival status and biopsy, self-reported symptoms, and laboratory markers of iron metabolism and fecal calprotectin level. The gingival and bowel inflammation was controlled with, strict adherence to a cinnamon- and benzoate-free diet combined with periodontal prophylaxis. At this point at the age of 17 years, the patient has avoided the use of immune modulatory treatments.