Search

Cutaneous tuberculosis (TB) makes up a small proportion of extrapulmonary TB. Studies from India report an incidence of 0.1% of all cases of extrapulmonary TB. Lupus vulgaris (LV) is a common form of TB which is usually seen in individuals who have been previously sensitized with Mycobacterium TB. Here, we are reporting a case of orofacial granulomatosis in a 21-year-old female which highlights the importance of histopathology in aiding diagnosis in such patients where multiple etiologies may be responsible for a particular clinical presentation, along with the atypical presentation of LV.

IntroductionCrohn’s disease (CD) is a chronic inflammatory bowel disease which can affect any area of the gastrointestinal tract, including oral tissues. The complex nature of this disease demands interdisciplinary management, especially when both intestinal and oral manifestations are present.CaseThis report presents the case of a 28-year-old male patient with oral, ileo-caecal and peri-anal CD managed jointly between Gastroenterology and Oral Medicine. Treatment with vedolizumab, an α4β7 integrin with gut-selective anti-inflammatory activity, resulted in excellent ileo-caecal disease control, but was ineffective in controlling oral manifestations. The absence of MAdCAM-1 expression in oral tissues, necessary for vedolizumab’s mechanism, meant that the drug’s anti-inflammatory effects were limited to the gut. This limitation led to worsening oral symptoms, necessitating concomitant azathioprine therapy to manage oral inflammation.ConclusionMultidisciplinary collaboration is important when managing CD patients with both oral and gut involvement in CD. Clinicians should be aware that vedoluzimab may be beneficial for intestinal CD, but does not target inflammation in oral tissues due to its gut-specific action. Good knowledge of the pharmacology and mechanism of action of drugs prescribed can aid decision making when prescribing for this group of patients and can limit the need for polypharmacy, often associated with an increased adverse effect profile.

Orofacial granulomatosis is an uncommon skin condition affecting the face and predominantly the lips, also referred to as cheilitis granulomatosa. We will be discussing a case of a 50-year-old female with a history of swollen lips for the past 3 years who had visited various hospitals and also had undergone multiple diagnostic tests with no conclusive diagnosis and was treated for the possible diagnoses of hemangioma and arterio-venous malformation with ineffective treatment results. Upon careful elicitation of details, we arrived at the diagnosis of orofacial granulomatosis and treated her with a combination therapy with resultant rapid regression of the lesion.

Orofacial granulomatosis is a rare disorder that is heterogeneously defined in the published literature. Herein, we describe a patient with orofacial granulomatosis with clinical and histologic evidence, discuss differential diagnoses, and offer clinical pearls for diagnosing and assessing this disorder. Our case provides support that orofacial granulomatosis is a distinct disorder as opposed to a sequela of other systemic granulomatous diseases. This information will aid dermatologists in decision making and diagnosing the disorder.

The objectives of the study were to (i) document refugee, immigrant and migrant (RIM) communities' knowledge, attitudes and beliefs (KABs) related to the Coronavirus disease (COVID-19) vaccine and (ii) identify best practices for developing and disseminating culturally and linguistically responsive health messaging addressing those KABs. Thirteen online focus groups (OFGs) in 10 languages were conducted. Each OFG was conducted in the participants' native language. OFGs were recorded, transcribed, translated and uploaded to qualitative software for coding. A thematic analysis was conducted. Results suggest that while there was some variation between different language groups (e.g. whether religious leaders were seen as trusted sources of information about COVID), there were also important commonalities. Most language groups (i) alluded to hearing about or having gaps in knowledge about COVID-19/the COVID-19 vaccine, (ii) reported hearing negative or conflicting stories about the vaccine and (iii) shared concerns about the negative side effects of the vaccine. There continues to be a need for health messaging in RIM communities that is culturally and linguistically concordant and follows health literacy guidelines. Message content about the COVID-19 vaccine should focus on vaccine importance, effectiveness and safety, should be multimodal and should be primarily delivered by healthcare professionals and community members who have already been vaccinated. [ABSTRACT FROM AUTHOR]

Inflammatory mucocutaneous conditions that affect the oral cavity are wide ranging with overlapping clinical and histological features. Clinically these can present with a range of appearances including lace to plaque like patches to erosive and ulcerative conditions. Many of these conditions are associated with systemic affects including gastrointestinal, ocular and dermatological. Early recognition is essential to ensure optimal patient care and appropriate referral to other specialists. This review will concentrate on lichenoid mucositis including lichen planus and erythema multiforme, the vesiculo-bullous conditions pemphigus and pemphigoid and the more common granulomatous conditions that present in the oral cavity. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Melkersson–Rosenthal syndrome (MRS) remains an enigmatic pathology due to an unknown etiology. Our report, of a 69‐year‐old man with MRS misdiagnosed for about 40 years, underlines the diagnostic difficulty of this condition. A holistic view of the patient, with a correct medical history investigation, are often decisive in the diagnosis of MRS. Melkersson–Rosenthal syndrome (MRS) is a rare disorder with a still unknown etiology. It is defined by three main symptoms, which are orofacial granulomatosis (OFG), facial palsy, and fissured tongue. It generally presents in young people, during the second or third decade, and its incidence in the entire population is about 1%. We focus our attention on a 69‐year‐old man who came to us with an important swelling of the upper lip. His anamnesis revealed that he suffered from a facial palsy four times in his life and at the physical examination we attested the presence of scrotal tongue. We suspected a misdiagnosed MRS and we searched the web in order to give him a diagnosis and a therapy. We found that OFG is the most common symptom of MRS and that it can show as a non complete form, where the three main symptoms cannot occur simultaneously. We also prescribed a therapy based on the use of topic steroids and antiviral, according to literature. After the positive response to the therapy and according to data found in the most recent literature, we can assume that our patient suffers from a misdiagnosed MRS for about 40 years. [ABSTRACT FROM AUTHOR]

Key Clinical Message Melkersson–Rosenthal syndrome (MRS) remains an enigmatic pathology due to an unknown etiology. Our report, of a 69‐year‐old man with MRS misdiagnosed for about 40 years, underlines the diagnostic difficulty of this condition. A holistic view of the patient, with a correct medical history investigation, are often decisive in the diagnosis of MRS. Abstract Melkersson–Rosenthal syndrome (MRS) is a rare disorder with a still unknown etiology. It is defined by three main symptoms, which are orofacial granulomatosis (OFG), facial palsy, and fissured tongue. It generally presents in young people, during the second or third decade, and its incidence in the entire population is about 1%. We focus our attention on a 69‐year‐old man who came to us with an important swelling of the upper lip. His anamnesis revealed that he suffered from a facial palsy four times in his life and at the physical examination we attested the presence of scrotal tongue. We suspected a misdiagnosed MRS and we searched the web in order to give him a diagnosis and a therapy. We found that OFG is the most common symptom of MRS and that it can show as a non complete form, where the three main symptoms cannot occur simultaneously. We also prescribed a therapy based on the use of topic steroids and antiviral, according to literature. After the positive response to the therapy and according to data found in the most recent literature, we can assume that our patient suffers from a misdiagnosed MRS for about 40 years.

Background: Orofacial granulomatosis (OFG) is an inflammatory disorder of the perioral region and oral cavity. Crohn's disease (CD) in conjunction with OFG (CD-OFG), has been suggested to constitute a phenotype of CD with distinct features at diagnosis. Aims: The aim of this project was to investigate whether the distinct phenotypic features of CD-OFG persist in the years following the initial diagnosis of CD. Methods: Clinical data were extracted from medical records covering the first 5 years post-diagnosis for a cohort of patients with CD-OFG, and were compared to those of references with CD without OFG. Results: The clinical characteristics of our cohort of patients with CD-OFG (N = 25) were evaluated in comparison to references with CD without OFG (ratio 1:2). Five years post-diagnosis, more patients with CD-OFG had a phenotype with perianal disease (cumulative incidence: 16/25, 64% vs 13/50, 26%, P = 0.002) and intestinal granulomas (cumulative incidence: 22/25, 88% vs 24/50, 48%, P = 0.0009) than patients in the CD reference group. The patients with CD-OFG were also more likely to have undergone perianal surgery (12/25, 48% vs 4/50, 8%, P = 0.0002). At the end of the observation period, more of the patients with CD-OFG were receiving combination therapy, i.e., immunomodulators and tumor necrosis factor antagonists, than those in the CD reference group (9/25, 36% vs 5/50, 10%, P = 0.01). Conclusion: The results support the notion that CD in conjunction with OFG represents a specific phenotype of CD that is characterized by frequent perianal disease, pronounced intestinal granuloma formation and a need for extensive therapy. [ABSTRACT FROM AUTHOR]

Orofacial granulomatosis (OFG) is an uncommon clinicopathological condition describing patients who have oral lesions characterized by persistent labial enlargement, oral ulcers and a variety of other orofacial features. Sarcoidosis is a systemic non-caseating granulomatous disorder of unknown etiology that may affect multiple organ systems. As a systemic disease, it is well documented in existing literature. However, the rarity and its often non-specific oral manifestation emphasize the need to reinforce the literature with more clinical case examples and draw additional attention to clinical features that general practitioners may encounter. In this report, a rare case is presented with definite association of sarcoidosis and orofacial granulomatosis with two years follow up after treatment. This report aims to expand the literature surrounding orofacial sarcoidosis and support oral and general health practitioners in recognizing its orofacial manifestations along with the clinical presentations that all led to a diagnosis of sarcoidosis in this case. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

ABSTRACT 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.

Orofacial granulomatosis is a condition that manifests clinically as painless labial enlargement, perioral and mucosal edema, oral ulcers, and gingivitis. It is characterized by non-necrotizing granulomatous inflammation of the oral and maxillofacial region. When the swelling only affects the lips, the pathology is called Miescher's granulomatous cheilitis; however, when it also causes facial paresis and lingua plicata, it is known as Melkersson-Rosenthal syndrome. We report a case that was successfully treated with a combination of a local (intralesional) steroid, a systemic antibiotic, and a systemic steroid. After 6 months of therapy, we observed improvement in gingival hyperplasia and buccal mucosa and lip edema. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

A 4‐year‐old girl was admitted to hospital with disturbance of balance. After being questioned, parents remembered an otitis with effusion 3 months earlier. CT‐scans revealed destruction of both temporal bones. Initial biopsy showed granulomatous, necrotic inflammation, which led to comprehensive differential diagnoses. A second tissue sample confirmed Langerhans cell histiocytosis. [ABSTRACT FROM AUTHOR]

Orofacial granulomatosis (OFG) is a rare syndrome that can occur in association with Crohn's disease (CD). The electronic medical record was searched for "OFG" and "CD." A total of 297 patients were identified, and relevant data were abstracted. Five patients met inclusion criteria. Most (80%) had ileocolonic CD with nonstricturing/nonpenetrating phenotype. The most common treatments included intralesional corticosteroids (4 patients), oral corticosteroids (3 patients), and anti-TNF agents, specifically infliximab (3 patients). Intralesional corticosteroid administration was followed by partial response in 3 patients, but recurrences were common following initial injection. One patient had no response to topical or oral corticosteroids, antihistamines, or oral antibiotics, but partial response to intralesional corticosteroids and infliximab. None of the patients had complete response to any therapies directed at this condition. OFG may be characterized by partial response, often requiring long-term therapy. Additional investigations into novel treatments may improve future clinical outcomes., (© 2024 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of The American College of Gastroenterology.)

Introduction: Sarcoidosis is a chronic inflammatory disease characterized by non-caseous necrotizing epithelial cell granulomas that can affect any organ. Ear, nose, and throat (ENT) involvement is rare. We report two cases of systemic sarcoidosis with ENT onset., Observation: A 39-year-old woman with no medical history, presented with right submaxillary lymphadenopathy, associated with a nasopharyngeal swelling on the nasopharyngoscopy. Lymph node biopsy revealed numerous granulomas with epithelioid and giant cells. The computed tomography (CT) showed multiple lymph nodes along with interstitial lung disease. The diagnosis of sarcoidosis with pulmonary, mediastinal, lymph node, and nasopharyngeal involvement was retained and the patient was treated with glucocorticoids with disappearance of the submaxillary swelling and of the lymph nodes on the CT. Another 39-year-old woman, with no medical history, presented to the ENT clinic with a three-month history of bilateral nasal obstruction. Nasal endoscopy revealed hyperemia of the nasal mucosa. She was treated with antibiotics without clinical improvement. CT scan of the cranial and facial bones revealed a focal bulge at the nasopharynx's posterior wall, associated with a mucosal thickening of maxillary, frontal, and ethmoid sinuses. A nasal and a lymph node biopsy were then performed, revealing granulomatous inflammation without caseous necrosis. The diagnosis of sarcoidosis with pulmonary, lymph node, and sinonasal involvement was established. The patient was treated with glucocorticoids with clinical improvement., Conclusion: Nasopharyngeal and sinus involvement may be rare in sarcoidosis but can be part of the initial clinical picture of the disease. Therefore, it should put the flea in the ear in case of ENT granulomatosis.

Abstract A 4‐year‐old girl was admitted to hospital with disturbance of balance. After being questioned, parents remembered an otitis with effusion 3 months earlier. CT‐scans revealed destruction of both temporal bones. Initial biopsy showed granulomatous, necrotic inflammation, which led to comprehensive differential diagnoses. A second tissue sample confirmed Langerhans cell histiocytosis.

Purpose: Crohn's disease (CD) is a chronic inflammatory bowel disease characterised by localised areas of nonspecific, noncaseating granulomas. Approximately 1/3 of CD patients develop extraintestinal manifestations in the course of their disease. This review focuses on oral manifestations of CD to understand if oral lesions could help clinicians in the diagnosis of systemic CD.Materials and Methods: Literature for the review was retrieved using PubMed Medline, Ebsco Library and Web of Science.Results: After a careful preliminary evaluation, only 43 articles were eligible for inclusion in the qualitative evaluation, whereas only 7 mentioned oral CD as the first sign of a systemic disease and were included in the quantitative evaluation.Conclusions: Oral manifestations of CD can be classified as specific and non-specific. The aetiology of oral CD seems to be linked to particular bacterial infections. Although the evidence from the literature is weak, it seems that in some cases the inspection of the mouth could assist in the diagnosis of a systemic Crohn's disease. [ABSTRACT FROM AUTHOR]

Skin conditions are a common reason for consulting primary health care. The public frequently expects their primary health care providers to be able to recognise and treat common skin conditions with confidence. Primary care providers must be able to identify ‘red flag’ skin conditions that require dermatology referral. However, diagnosis is often delayed due to the non-specific clinical presentation, low incidence, slow evolution and long-term history of such lesions. We report the case of a patient with extramammary Paget’s disease that presented to her primary care provider and subsequently underwent a wide local excision.

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. [ABSTRACT FROM AUTHOR]

This article highlights cheilitis granulomatosa (CG) of the upper lip in a 6-year-old female child with neglected oral hygiene. CG is a rare, idiopathic, chronic inflammatory disorder presenting as a diagnostic dilemma due to a wide array of etiological factors. There is no standard treatment and often unpredictable therapeutic responses and spontaneous recurrences are reported. This article emphasizes on providing specialized dental treatment for active oral infections as the first line of therapy, especially in pediatric patients as it may turn out to be a major shot in the arm. [ABSTRACT FROM AUTHOR]

Background: Racial variation exists in the incidence of orofacial granulomatosis (OFG). The epidemiology and clinical characteristics of OFG in Asian countries are poorly described. Objective: To describe the epidemiologic and clinical features of OFG in China from data collected on chronic odontogenic infection and studied in actual practice regarding the long-term outcome of OFG patients receiving different treatments. Methods: Data on demographics, medical history, chronic odontogenic infection, and the extent of disease were collected, and long-term outcomes after the end of treatments were evaluated. Results: Of the 165 OFG patients, 118 (71.5%; 95% CI 64.6%-78.5%) had a chronic odontogenic infection. There was a variety of difference between OFG with and without chronic odontogenic infection. Approximately 98.3% (95% confidence interval 94.8%-100%) of OFG patients with chronic odontogenic infection who received dental treatment showed a marked response, of whom 31 patients (53.4%; 95% confidence interval 40.2%-66.7%) had complete remission. Limitations: Endoscopic investigations were not performed for most of the patients, and more detailed data were not collected, which might have demonstrated additional systemic problems. Conclusions: OFG with chronic odontogenic infection is the major clinical pattern of OFG in China, which may be a subtype of OFG. Dental treatment should necessarily be the preferred first-line therapy for such patients.

Abstract Background Miescher’s cheilitis granulomatosa (MCG) is a rare chronic inflammatory disease and is known as the monosymptomatic clinical form of Melkersson-Rosenthal syndrome (MRS). It is characterised by swelling of one or both lips and more frequently affects the upper lip. Histopathological findings show the presence of numerous inflammatory infiltrates and granuloma formations. Pharmacological treatments and surgery have provided results that are positive yet insufficiently stable in the long term. The clinical case described is of a 68-year-old female patient with a diagnosis of MCG of the upper lip. Case presentation The patient was diagnosed and treated at the Oral Medicine and Oral Pathology outpatient clinic of Maxillofacial and Odontostomatology Unit, Fondazione Cà Granda IRCCS Ospedale Maggiore Policlinico. The patient was recommended localised treatments of photobiomodulation (PBM) using a diode laser with a 635 nm and 980 nm dual-wavelength (λ) approach, a 600-micron fibre, and a handpiece with a 1-cm-diameter lens at 300 mW. Three treatments a week were administered for four weeks for a total of 12 treatment sessions (T 1–T 12). After that, the patient had a long follow-up period of about 2 years. The therapeutic results were clear from the initial stages of treatment. There was an immediate, gradual, and consistent reduction in labial swelling. A reduction in the size of the lip by about 35% at T 10–T 12 was observed, returning the size and volume of the upper lip within the normal clinical range. The painful symptoms subsided after the seventh treatment (T 7). The histopathological check at 3 months and the follow-up in particular confirmed the disease was in remission with satisfactorily stable treatment results. Moreover, the patient did not use any other treatments on the area from the early laser treatments through to the end of the follow-up period. Conclusions Our experience describes a clinical case of MCG treated with PBM and effectively resolved with a reduction of the lip swelling. The real success of the treatment emerged over time, showing that the tissue healing was stable. In absence of any collateral phenomena, this confirms the effective and documented therapeutic potential of PBM for chronic inflammatory infiltrates.

Ronald Brown,1 Andre Farquharson,1 Gail Cherry-Peppers,2 Leslie Lawrence,3 Donna Grant-Mills2 1Department of Oral Diagnosis & Radiology, Howard University College of Dentistry, Washington, DC, USA; 2Department of Restorative Dentistry, Howard University College of Dentistry, Washington, DC, USA; 3Department of Pediatric Dentistry, Howard University College of Dentistry, Washington, DC, USACorrespondence: Ronald BrownDepartment of Oral Diagnosis & Radiology, Howard University College of Dentistry, 600 W Street, NW, Washington, DC 20059, USATel +1 202-806-0020Email rbrown@howard.eduAbstract: A case of a 19-year-old female patient is presented to a private practice dental clinician with swelling of the lower lip and inflammation of the anterior dorsal tongue. The patient presented with moderate oral pain as well as abdominal pain. The lesions were biopsied and noted for a granulomatous histopathologic appearance. The patient reported a history of using cinnamon as a flavoring agent. The lesions resolved within two weeks after the biopsy procedures and topical steroid therapy. The lesions were diagnosed as cheilitis granulomatosa/orofacial granulomatosis. The patient has remained lesion free as of the three-year follow-up. Etiologic, diagnostic and therapeutic issues related to this relatively rare condition of cheilitis granulomatosa/orofacial granulomatosis are discussed.Keywords: cheilitis granulomatosa, orofacial granulomatosis, cinnamon

Sumeet R Dhawan,1 Arushi G Saini,2 Pratibha D Singhi2,3 1Department of Pediatrics, Maharishi Markandeshwar Institute of Medical Sciences and Research, Mullana, Ambala, 133207, India; 2Pediatric Neurology Unit, Department of Pediatrics, Postgraduate Institute of Medical Education and Research, Chandigarh 160012, India; 3Medanta, The Medicity, Gurgaon, Haryana, IndiaCorrespondence: Pratibha D Singhi Medanta, The Medicity, Gurgaon, Haryana, IndiaTel +91 8168186894Email doctorpratibhasinghi@gmail.comAbstract: Melkerrson-Rosenthal syndrome is a rare disorder of unknown aetiology and characterized by the triad of oro-facial edema, facial nerve palsy, and furrowing of the tongue. Two or more of the above are essential for making a clinical diagnosis. The mainstay of treatment is corticosteroids. Intralesional triamcinolone acetonide may be used for the treatment of oro-facial edema. Another treatment option for oro-facial edema includes intralesional betamethasone, along with oral doxycycline. The review discusses the management strategies in Melkersson-Rosenthal syndrome.Keywords: facial palsy, Bell’s palsy, facial edema, lip edema, orofacial granulomatosis, recurrent facial palsy, fissured tongue, lingua plicata

Wiesenfeld described orofacial granulomatosis (OFG) as a group of noncaseating granulomatous disorders affecting the different parts of the body including oral and maxillofacial region, which can have variable etiology. The involvement of oral and maxillofacial region can be exclusive or primary before the involvement of other organs. OFG is a multifactorial clinicopathologic disorder, and its clinical manifestation mimics the various systemic conditions making its diagnosis tough for a dentist. Delay in diagnosis and evaluation of OFG may cause indurated, swollen lips that may compromise cosmetic face value, speaking, and eating functions, and other possible systemic granulomatous disorders can be missed. Here, we present a case of OFG with tooth-associated infection without any recognizable systemic cause. This article presents the diagnostic challenges that a dentist may face in diagnosing such cases, and thus treatment planning should be of the multidisciplinary approach. Regular follow-up is very essential for proper care and management.

We describe a female child in the first decade patient admitted to a children's hospital for lip swelling and constipation who was ultimately diagnosed with Crohn's disease. Her lip swelling resolved after the initial immunosuppression for her Crohn's disease. In this article, we discuss the pretest probability of cutaneous findings associated with inflammatory bowel disease in predicting a diagnosis of Crohn's disease in the absence of intestinal symptoms. Cutaneous findings including linear immunoglobulin A bullous dermatosis, pyoderma gangrenosum, lichen spinulosus, and epidermolysis bullosa acquisita are discussed.

Introduction: Granulomatous cheilitis (GC) is a rare idiopathic inflammatory disorder characterized by chronic persistent swelling of the lips. Despite a lingering controversy, it remains linked to orofacial granulomatosis and Melkersson–Rosenthal syndrome (MRS). Aim: To systematically review all cases of GC reported hereto from India with the three being reported now, analyzing patients'/disease characteristics, associated diseases, and treatment protocol. Materials and Methods: Using biomedical search engines such as PubMed, Scopus, and Google Scholar, a systemic review of cases/case series reported from India (1994–2018) was undertaken, and data on patients'/disease characteristics, association with other diseases, and treatment extracted discussed and analyzed without subjecting to statistical analysis due to the small population size. Results: The mean age at presentation of these cases (n, 73: Single, 39: Participants of 6 series, 31 plus presently reported, 3) was 29.36 years and at onset was 26.62 years. There was female preponderance of 1.8. Complete triad of MRS was recorded in 8 (10.95%), and oligosymptomatic forms were as follows: GC + facial palsy, 5 (6.85%); GC + fissured tongue, 4 (5.48%); and facial palsy + fissured tongue, 1 (1.37%). Monosymptomatic GC was seen in 55 (75.34%) and upper lip involvement in 29 (39.72%). Conditions associated with GC included Down syndrome, rosacea, granulomatous vulvitis, and pregnancy. None of the cases were associated with Crohn disease and sarcoidosis. Intralesional triamcinolone acetonide (33, 45.21%) was the most common modality of treatment. Conclusion: Cases of GC from India over the past quarter of a century were reported rarely and showed female preponderance. This chronically recurrent entity with variable severity lacks larger studies and randomized controlled trials for optimum treatment.

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.

Melkersson-Rosenthal Syndrome (also termed “Miescher-Melkersson-Rosenthal Syndrome”) or (MRS), is a rare neurocutaneous disorder characterized by the clinical triad of recurring facial nerve paralysis, swelling of one or both lips and fissural tongue. We report a patient with Melkersson-Rosenthal Syndrome initially referred to Dermatology Department as angioedema. The diagnosis and treatment procedures were presented with special emphasis to the clinical features of this rare condition.

Copyright of Dermatologie Pro Praxi is the property of SOLEN sro and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

The association between cheilitis granulomatosa and dental infections (dental caries and apical periodontitis) is still not well understood. Herein, we aimed to investigate the association in large hospital cases with cohort controls. Cheilitis granulomatosa cases (n = 181) were retrieved from Peking University Hospital of Stomatology and age‐ and sex‐matched to controls (n = 181). The χ2‐test, Student's t‐test, and Mann–Whitney U‐test were used to compare the differences between groups. The χ2‐test and odds ratio were used to verify if there was an association and risk relationship. The results showed that both dental caries and apical periodontitis were associated with cheilitis granulomatosa (p < 0.001). Individuals with cheilitis granulomatosa had approximately a twofold increased frequency of dental caries than those without cheilitis granulomatosa (104/181, 57.5% vs. 53/181, 29.3%) (p < 0.001). The odds ratio of dental caries occurring in the case group compared to the control group was 3.211. The frequency of apical periodontitis in patients with cheilitis granulomatosa was significantly greater than in those without cheilitis granulomatosa (109/181, 60.2% vs. 28/181, 15.5%) (p < 0.001). The odds ratio was 8.272. Moreover, apical periodontitis was also locationally related to cheilitis granulomatosa (p < 0.001). Collectively, our study showed that the foci of dental infections are associated with cheilitis granulomatosa, suggesting that proper treatment of focal teeth may be important in the management of cheilitis granulomatosa. [ABSTRACT FROM AUTHOR]

OBJECTIVE: Orofacial granulomatosis (OFG) is a relatively rare entity. The information pertaining to it is accessible to patients and care-givers on the internet in a scarce quantity. The aims of the current study were to evaluate quality, readability, understandability and actionability of information regarding the disease-orofacial granulomatosis-available to the public on the internet. The purpose of the study was to evaluate ease of access and understandability of language available for individuals affected by OFG. Although the incidence of the disease is very low in Pakistan, patients with symptoms of OFG rarely report to clinics due to social stigma. METHODOLOGY: A vivid selection process was chosen for the study. The study was conducted online on 'google scholar' website. Four different search terms were used 'ofg disease', 'orofacial granulomatosis', 'cheilitis granulomatosa' and 'Melkersson Rosenthal Syndrome' to seek information on orofacial granulomatosis. All of this was done during July, 2020 to October 2020. The first 100 results from each term were shortlisted and evaluated further. Exclusion criteria was used and several repetitive sites, non-functional links, sites containing content irrelevant to the search were excluded. This resulted in 58 websites relevant to the search that were then categorized according to affiliation, specialisation, content type as well as content presentation. Three grading assessments were utilized to assess the quality of this online information; the Journal of the American Medical Association (JAMA) benchmarks, the Patient Education Materials Assessment Tool for Printable Materials (PEMAT-P) and the presence of Health on the Net (HON) seal. In order to assess the readability of the content in the websites, the Flesch Reading Ease Score (FRES) and Simple Measure of Gobbledygook (SMOG) were used. RESULTS: The overall quality of online information on orofacial granulomatosis is difficult to comprehend and act upon as assessed by the PEMAT. The HON seal was visible on only 5 (9%) websites. In terms of readability, only one (2%) website was fairly easy to read. CONCLUSIONS: The online information on orofacial granulomatosis is qualitatively poor and does not serve its purpose in true sense. There is a need to devise better, high quality online readable information for patients and the public to understand. [ABSTRACT FROM AUTHOR]

Background: Orofacial granulomatosis (OFG) is characterized by granulomatous inflammation of the soft tissues of maxillofacial region. We explored OFG patients from 10 different Italian centers and summarized the most recent literature data. Methods: A review of patients with OFG was carried out. An extensive online literature search was performed to identify studies reporting diagnosis and management of OFG. Results: Thirty‐nine patients were recruited between January 2018 and February 2020. Most of them (97.4%) displayed involvement of the lips, and 28.2% suffered from Melkersson‐Rosenthal syndrome. Two patients received diagnosis of CD and one patient of sarcoidosis, suggesting secondary OFG. Oral aphthosis and cervical lymphadenopathy were also described. The mean diagnostic delay was 3.4 years. Histological evaluation was performed in 34/39 patients (87.2%); non‐caseating granulomas were found in 73.5% of them. Neurological symptoms (28.2%), gastrointestinal symptoms in absence of overt inflammatory bowel disease (IBD) (20.5%), and atopy (35.9%) were also identified. Therapeutic approaches varied among the centers. Steroids (51.3%) were used with good or partial results. Anti‐TNF‐α and anti‐IgE monoclonal antibodies were used in 6 (15.4%) and 1 (2.6%) patients, respectively, with variable results. Surgery was the choice for 2 patients with good response. Conclusions: OFG is a rare and neglected disease showing multiple clinical phenotypes. While early diagnosis is crucial, management is difficult and highly dependent on the expertise of clinicians due to the lack of international guidelines. There is a need to establish registry databases and address challenges of long‐term management. [ABSTRACT FROM AUTHOR]

Background: Melkersson–Rosenthal syndrome (MRS) is a rare disease characterized by the triad of granulomatous cheilitis, fissured tongue, and facial paralysis. Publications concerning large series are rare in the literature. Objectives: To describe the clinical and histopathological characteristics of patients with complete and oligosymptomatic forms of MRS. Methods: A retrospective records review was performed for the diagnoses of Melkersson-Rosenthal syndrome, granulomatous cheilitis, and orofacial granulomatosis at oral Diseases Clinic of the Department of Dermatology, University of São Paulo, Brazil (2003, 2017). Results: A total of 51 patients were included, mean age at presentation 35.69 years. Four patients were younger than 18 years. The complete triad of was observed in 10 patients. The rare findings of granulomatous blepharitis, gingivitis and palatitis are presented. Comorbidities included Crohn's disease (5 patients), migraine headaches (1 patient) and convulsions (2 patients). Granulomatous inflammatory infiltrate was detected in 31 biopsies. Medical therapies included included oral and intralesional steroids, thalidomide, dapsone, azathioprine, tetracycline, methotrexate, and surgery, with variable responses. Conclusions: Our report meant to draw attention to the clinical spectrum of this rare disorder, mainly to oligosymptomatic forms and rarer presentations. [ABSTRACT FROM AUTHOR]

Background: To systematically review all cases of orofacial granulomatosis (OFG) and evaluate the association between OFG and Crohn disease (CD). Summary: This review was conducted according to PRISMA guidelines and a search of the PubMed, MEDLINE, and Embase databases, and the Cochrane Library in March 2020, using keywords and MeSH terms associated with "orofacial granulomatosis," "Crohn disease," and their variants, with no language restrictions and across all age groups. All relevant articles were accessed in full text. Single case reports and articles on sarcoidosis, allergy, ulcerative colitis, and infectious diseases were excluded from the analysis. Results: We retrieved 507 reports on OFG. The mean age at onset was 23.3 years (range 2–89 years). A total of 240 (47.3%) females and 267 (52.6%) males were included. CD was present in 93 children aged <16 years (68.3%) and in 43 adults (31.9%). In most cases, the OFG appeared before the CD. The most common clinical manifestations were intraoral mucosa abnormalities (n = 251; 49.5%), lower-lip swelling (n = 249; 49.1%), upper-lip swelling (n = 227; 44.7%), and gingivae (n = 193; 38.7%). Patients with concurrent CD were more likely to experience involvement of the buccal sulcus. Key Messages: OFG presents primarily as a solo entity. The OFG that was associated with CD was present in 93 children aged under 16 years (68.3%) and in 43 adults (31.9%). Childhood onset of OFG carries with it a higher risk of developing CD. [ABSTRACT FROM AUTHOR]

Persistent soft tissue enlargement in the orofacial region is often considered a feature of a chronic disease, termed as orofacial granulomatosis (OFG). Systemic diseases like crohn's, inflammatory bowel syndrome present OFG as their oral manifestation, but it may also be seen in the absence of these diseases. To the best of the authors' knowledge and literature search, this is the first case of OFG with gingival deformity related to cigarette smoking. A 25-year-old male patient presented with chief complaint of swelling on his upper lip, increase in the size of gingiva and hypersensitivity in the maxillary anterior teeth region. After thorough history of disease initiation in response to cigarette smoking, clinical examination, histopathological findings, the diagnosis of OFG associated with cigarette smoking was made. Treatment included cessation of cigarette smoking, internal bevel gingivectomy, with proper medication. Single treatment modality may not be effective in such cases. Therefore, early and precise diagnosis with sequential treatment including elimination of etiological agent, anti-inflammatory drugs or even plastic surgeries may be required for improving patient's health, appearance and comfort. [ABSTRACT FROM AUTHOR]