Orofacial Granulomatosis Research Articles

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.

Gingival orofacial granulomatosis clinical and 2D/3D microscopy features after orthodontic therapy: a pediatric case report

Gingival orofacial granulomatosis clinical and 2D/3D microscopy features after orthodontic therapy: a pediatric case report

Diagnostic delay in autoimmune oral diseases.

Autoimmune diseases (ADs) affect about 5% of the general population, causing various systemic and/or topical clinical manifestations. The oral mucosa is often affected, sometimes as the only involved site. The misdiagnosis of oral ADs is an underreported issue. This narrative review focuses on diagnostic delay (DD) in oral ADs (oral lichen planus [OLP], oral Pemphigus Vulgaris, mucous membrane pemphigoid, oral lupus erythematosus, orofacial granulomatosis, oral erythema multiforme [EM], and Sjogren syndrome). Extensive literature research was conducted via MEDLINE, Embase and Google Scholar databases for articles reporting the time spent to achieve the correct diagnosis of oral ADs. Only 16 studies reported DD in oral ADs. Oral autoimmune vesiculobullous diseases are usually diagnosed after 8 months from the initial signs/symptoms, the Sjogren Syndrome diagnosis usually requires about 73months. No data exist about the DD in OLP, oral lupus erythematosus, orofacial granulomatosis, and oral EM. The diagnosis of oral ADs can be difficult due to the non-specificity of their manifestations and the unawareness of dentists, physicians, and dental and medical specialists about these diseases. This can lead to a professional DD and a consequential treatment delay. The delay can be attributed to the physicians or/and the healthcare system (Professional Delay) or the patient (Patient's Delay).

Macroheilitis as a manifestation of Melkersson – Rosenthal syndrome

Melkersson – Rosenthal syndrome (SMR) is a disease manifested by a triad of symptoms: swelling of the lips and/or face, paralysis of the facial nerve, scrotal tongue. In clinical practice, the monosymptomatic variant of SMR is more common. The disease can develop at any age, but most cases occur at 25–40 years with a 2-fold predominance of women among patients. In children, SMR is more often observed at the age of 7 to 12 years, and the smallest child is described at the age of 22 months. The causes of the Melkersson – Rosenthal syndrome have not been definitively clarified. There are many hypotheses about the pathogenesis of SMR, the main of which are allergic, angioedema, infectious and genetic, immunological dysfunction. There are various historically established terms: Mischer’s granulomatous cheilitis, orofacial granulomatosis, SMR, which are regularly used as synonyms in the available literature. To date, SMR remains a diagnosis of exclusion, which is accompanied by the establishment of controversial diagnoses in practical healthcare. In most cases, the treatment of SMR is ineffective. Mainly systemic glucocorticosteroids, antibiotics, antihistamines, immunosuppressive drugs are used, upon discontinuation of which the disease often recurs. In this publication, we present two clinical cases of SMR. The interest of the first case lies in the presence of the entire group of clinical symptoms and the achievement of a positive treatment result. The second case is characterized by the achievement of long-term remission as a result of combination therapy with betamethasone, azithromycin and ketotifen. An overview of successful treatment options is also presented.

Frequent Occurrence of Perianal Disease and Granuloma Formation in Patients with Crohn’s Disease and Coexistent Orofacial Granulomatosis

BackgroundOrofacial 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.AimsThe 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.MethodsClinical 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.ResultsThe 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).ConclusionThe 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.

Intralymphatic histiocytosis-like changes in orofacial granulomatosis.

This report provides rare histopathological findings of intralymphatic histiocytosis in orofacial granulomatosis involving the lower lip and unilateral upper eyelid. The number of patients with findings of intralymphatic histiocytosis in the periorbital regions is small. Here, the underlying pathogenesis of intralymphatic histiocytosis is discussed.

Factors Participating in the Occurrence of Inflammation of the Lips (Cheilitis) and Perioral Skin

Lip inflammation may manifest as mainly reversible cheilitis, mainly irreversible, or cheilitis connected to dermatoses or systemic diseases. Therefore, knowing a patient’s medical history is important, especially whether their lip lesions are temporary, recurrent, or persistent. Sometimes temporary contributing factors, such as climate and weather conditions, can be identified and avoided—exposure to extreme weather conditions (e.g., dry, hot, or windy climates) may cause or trigger lip inflammation. Emotional and psychological stress are also mentioned in the etiology of some lip inflammations (e.g., exfoliative cheilitis) and may be associated with nervous habits such as lip licking. To better manage cheilitis, it is also helpful to look for potential concomitant comorbidities and the presence of related diseases/conditions. Some forms of cheilitis accompany dermatologic or systemic diseases (lichen, pemphigus or pemphigoid, erythema multiforme, lupus, angioedema, xerostomia, etc.) that should be uncovered. Occasionally, lip lesions are persistent and involve histological changes: actinic cheilitis, granulomatous cheilitis, glandular cheilitis, and plasmacellular cheilitis. Perioral skin inflammation with simultaneous perioral dermatitis can have various causes: the use of corticosteroids and cosmetics, dysfunction of the skin’s epidermal barrier, a contact reaction to allergens or irritants (e.g., toothpaste, dental fillings), microorganisms (e.g., Demodex spp., Candida albicans, fusiform bacteria), hormonal changes, or an atopic predisposition. Epidermal barrier dysfunction can worsen perioral dermatitis lesions and can also be related to secondary vitamin or mineral deficiencies (e.g., zinc deficiency), occlusive emollient use, sunscreen use, or excessive exposure to environmental factors such as heat, wind, and ultraviolet light. Current trends in research are uncovering valuable information concerning the skin microbiome and disruption of the epidermal barrier of persons suffering from perioral dermatitis. Ultimately, an effective approach to patient management must take all these factors and new research into account.

A RARE CAUSE OF LIP SWELLING

A 14-year-old, previously healthy, boy presented with a one-month history of persistent swelling of the lower lip. There were no associated symptoms or identifiable inciting events. Physical examination showed soft and painless swelling of the lower lip, particularly on the left side (Figure 1). Two months later, he presented to consultation with permanent lip swelling, weight loss, abdominal pain and vomiting. Initial bloodwork revealed increased inflammatory markers and calprotectin. Gastro-intestinal endoscopies displayed superficial erosions of the colon and esophagus. Mucosal biopsies showed noncaseating transmural granulomas and lip biopsies revealed giant cell granulomas without necrosis. The diagnosis of orofacial granulomatosis associated with Crohn’s disease was confirmed. The patient was treated with azathioprine and mesalamine, with good response. Orofacial granulomatosis is a rare disorder characterized by facial or lip swelling and can be related to systemic disease [1]. Crohn’s disease presenting as orofacial granulomatosis is very rare in the pediatric population and it can precede the intestinal symptoms of Crohn Disease by months or years [2,3]. Therefore, it is important to have a low threshold of suspicion and carry out persistent clinical surveillance for the development of additional signs and symptoms [2].

Treatment of Orofacial Granulomatosis—9-Month Follow-up: A Case Report

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.

Small lymphocytic lymphoma presenting as chronic diffuse lip swelling.

Although rare, small lymphocytic lymphoma can present as chronic lip swelling and papules, thus mimicking the features of orofacial granulomatosis, a chronic inflammatory disorder characterized by subepithelial noncaseating granulomas, or papular mucinosis, characterized by localized dermal mucin deposition of mucin. When assessing lip swelling, one must carefully consider the clinical clues and have a low threshold to perform a diagnostic tissue biopsy, preventing delays in treatment or progression of the lymphoma.

APPROACHING THE STIFF UPPER LIP: A CASE OF PSEUDOANGIOEDEMA DIAGNOSED AS OROFACIAL GRANULOMATOSIS

<h3>Introduction</h3> Angioedema describes transient swelling in the deeper layers of the skin and mucosa, broadly categorized as histaminergic or bradykinin-mediated. Allergists should also be familiar with conditions that mimic angioedema to hasten proper management and avoid negative impact from misdiagnosis. We describe a case of one such condition, Orofacial granulomatosis with pyostomatitis vegetans, involving disfiguring inflammation of the orofacial soft tissue and often associated with comorbid inflammatory bowel disease. <h3>Case Description</h3> A 61-year-old female with a history of dysphagia, COPD, Esophageal stenosis and stroke was referred to Allergy for ten years of recurrent facial and lip swelling. ER visits resulted in multiple rounds of corticosteroids with variable success. Antibiotics for presumed cellulitis, epinephrine, high dose antihistamines and bradykinin antagonists (icatibant) were unsuccessful. Work up included normal complement studies (C3, C4, C1q, C1 esterase quantity/function). After being lost to follow up, the patient re-presented with firm, fixed lip swelling (figure 1), pustules of the buccal mucosa, dysphagia and diarrhea, prompting hospital admission. Buccal biopsy demonstrated pathology suggestive of pyostomatitis vegetans, a rare inflammatory dermatosis strongly associated with IBD. Labs demonstrated high titer positivity for <i>saccharomyces cerevisiae</i> antibody (ASCA) IgG and IgA, with a 95% specificity for IBD, particularly ulcerative colitis. She is currently being trialed on empiric anti-TNF therapy with adalimumab. <h3>Discussion</h3> This is a rare case of pseudoangioedema from Orofacial granulomatosis with pyostomatitis vegetans in the setting of suspected inflammatory bowel disease. The progressive nature and complexity of this disease underscore the importance of Allergists being familiar with disorders that mimic angioedema.

AN UNCOMMON CAUSE OF LIP SWELLING

<h3>Introduction</h3> Angioedema is a common presentation in allergy clinic. Typical etiologies include mast cell mediated, bradykinin mediated, and idiopathic angioedema. It is important to consider less common causes and conditions that masquerade as angioedema. <h3>Case Description</h3> A 14-year-old male presented with persistent lower lip swelling. Medical history was notable for asthma, urticaria, allergic rhinitis, atopic dermatitis, and constipation. Examination showed asymmetric lower lip swelling without involvement of the tongue, uvula, or other area of the face. Laboratory work-up for suspected angioedema revealed normal CBC, elevated ESR, and normal C1 esterase inhibitor and C1 function. He tried cephalexin, mupirocin, cetirizine 20mg daily, montelukast, prednisone 50mg for 5 days and elimination of all product use on lips except for Vaseline without resolution of angioedema. A lip biopsy was obtained which revealed granulomatous cheilitis. The patient was referred to gastroenterology. Colonoscopy demonstrated granulomatous colitis and ileitis consistent with Crohn's Disease. Lip swelling resolved with adalimumab. <h3>Discussion</h3> While allergic and immunologic pathology are common causes of angioedema, this case encourages consideration of other etiologies of lip swelling, such as inflammatory bowel disease. Other diagnoses to consider include sarcoidosis, infections, miescher's cheilitis, and autoimmune conditions.

S2706 Orofacial Granulomatosis in Crohn's Disease

Introduction: Extra-intestinal manifestations (EIMs) of inflammatory bowel disease (IBD) are rare complications that affect roughly 6-25% of IBD patients. Orofacial granulomatosis (OFG) is a rare manifestation that does not have reliable epidemiologic data and is characterized by orofacial swelling with non-caseating granulomas seen on pathology. Case Description/Methods: A 23-year-old male with a history of Crohn’s colitis presented with loose stools and hematochezia for 7 months and 1 year of left sided lip swelling. He was diagnosed with Crohn’s disease at age 15 and was maintained on oral mesalamine and 6-mercaptopurine (6-MP). At age 16, the patient was evaluated for supraglottic edema. Urgent tonsillectomy was performed, with pathology revealing necrotizing and non-caseating granulomas. He recovered without complication. He achieved endoscopic remission at age 18 and his medications were discontinued. Unfortunately, he was lost to follow-up. During this subsequent presentation, his vitals were within normal limits. Exam was notable for left-sided lip and cheek swelling without mass or lesion. Bloodwork was notable for an ESR of 19 and CRP of 7.5. Lip biopsy showed normal squamous epithelium and non-caseating granulomas, consistent with OFG. Patient was treated for a Crohn’s flare with oral prednisone as a bridge to 6-MP. His OFG was treated with intralesional corticosteroids without relief and has on-going outpatient follow up. His current treatment plan includes further cycles of steroid injections before considering alternative therapies. His most recent colonoscopy showed endoscopic and histologic remission of his luminal Crohn’s while on infliximab. (Figure) Discussion: OFG is frequently seen as a manifestation of a systemic condition such as IBD. OFG is diagnosed by biopsy and clinical suspicion. OFG specific treatments include topical steroids and calcineurin inhibitors. Recent data suggests that a cinnamon/benzoate free diet may provide modest benefit to patients. Adequate treatment of underlying IBD is equally important. This patient has the classic symptoms and histopathologic findings of OFG and likely had undiagnosed OFG for many years given his tonsillar pathology years prior. Despite treatment, this patient has persistent and refractory symptoms. This case highlights a classic presentation of a rare IBD extra-intestinal manifestation and demonstrates how EIMs can occur even with well controlled IBD.Figure 1.: Orofacial Granulomatosis: Lip and Cheek Swelling.

P40: Histopathological features of orofacial granulomatosis with and without underlying gut Crohn disease

P40: Histopathological features of orofacial granulomatosis with and without underlying gut Crohn disease

OROFACIAL GRANULOMATOSIS

Introduction:- Orofacial granulomatosis (OFG) is a disease entity with recurrent or persistent edema within the orofacial region. It frequently manifests as persistent and/or recurrent upper and/or lower labial lip and gingival enlargement. Histological features are non-necrotizing granulomas form in the affected tissues in the absence of any recognized systemic disease. The denite etiology of OFG is unidentied.1 Numerous theory have been recommended, including infection, genetic predisposition, and allergy.2-6 This report is a remarkable case ofCase report:- orofacial granulomatosis which presented as a recurrent severe generalized gingival enlargement in a eleven year-old child. There was no family history and no data of an underlying allergic or systemic cause. Oral prophylaxis showed mild alleviation of symptoms. Diagnosis ofConclusion: - orofacial granulomatosis is often difcult. OFG is cured by appropriate treatment.

Disease-related burden and long-term outcome in orofacial granulomatosis: observations from a large single-centre cohort.

There is a lack of standardized treatment recommendations for orofacial granulomatosis, a chronic inflammatory condition aetiologically related to Crohn disease. To assess clinical baseline parameters and treatment strategies, we retrospectively analysed 61 consecutive cases from our institutional database. Disease-related functional/psychological impairment and long-term outcomes were descriptively evaluated using a standardized self-reporting questionnaire. The median age of patients was 45 (7-77) years. Oral steroids were given in 41.0% of cases, but only produced short-term disease control, while response to steroid-sparing agents was inconsistent. Only a minority of patients reported relevant disease-related functional impairment in eating (21.7%) or speaking (4.3%), but the majority perceived psychological distress due to the cosmetic aspects of the disease (69.6%), comments from others (65.2%) and/or general anxiety/insecurity (73.9%). Regardless of the initial treatment, long-term outcomes after 71 months (range 7-304 months) were beneficial, with most patients being in complete remission (52.2%) or reporting only mild residual swelling (43.5%).

Current treatment modalities in granulomatous cheilitis.

Granulomatous cheilitis (GC) is a poorly understood disorder of the labial mucosa, which is a subtype under an umbrella term "orofacial granulomatosis." Several treatment modalities have been tried in the management of GC with suboptimal to optimal results. The objective of this article is to discuss various treatment modalities, whether medical or surgical, that have been tried for the management of GC with success. A comprehensive literature search was performed to screen articles related to the successful treatment of GC. Articles published in journals indexed in PubMed were searched along with those in Google Scholar. There was no availability of systematic review or meta-analysis on treatment of GC in the literature search. Recent data has been collected and consolidated from various case reports and case series to provide a concise overview of various treatments applied in GC. Various treatment modalities have been used in GC including glucocorticoids, antibiotics, immunomodulatory, and biological agents, surgical debulking, and laser therapy. No treatment modality has provided a predictable success. Intralesional corticosteroid therapy has been used more frequently either alone or in combination with other modalities.

Granulomatous cheilitis in a patient after SARS-CoV-2 infection treated with antibiotics: a case report

Granulomatous cheilitis or Miescher's cheilitis is a rare granulomatous disorder defined by recurrent lip swelling or edema of other facial soft tissues. Histopathology shows non-caseous granulomas and multinucleated giant cells. The exact etiology is unknown, although genetic background, immunological irregularities, and systemic or infectious diseases contribute to the onset of disease. There are no treatment guidelines. The usual treatment options include systemic or intralesional corticosteroids, a spectrum of antibiotics, and immunosuppressants. A 63-year-old patient presenting with lip swelling and simultaneous swelling of other facial soft tissues was diagnosed with granulomatous cheilitis. The symptoms occurred 3 weeks after SARS-CoV-2 infection. Initial treatment with systemic corticosteroids and antihistamines was inadequate. Here we report successful treatment with a combination of doxycycline and metronidazole.

The Possible Link between Cheilitis Granulomatosa and Oral Health?

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.