Management of Cheilitis Granulomatosa with Pica Disorder: A Rare Case Report.

Aim: To report a rare case of cheilitis granulomatosa (CG) with pica disorder presented as chronic persistent lip swelling with an unusual eating disorder. Background: Cheilitis granulomatosa or Miescher's cheilitis is a rare, persistent, or recurring nontender chronic painless swelling of one or both the lips due to granulomatous inflammation, which was first described by Miescher in 1945. It can present as a monosymptomatic form or as a part of other entities of Melkersson-Rosenthal syndrome (MRS). Case description: A 14-year-old female patient presented with persistent diffuse swelling of the upper lip for 1 year. She also had an unusual habit of eating clay, mud, and potentially harmful items like flakes of paint for 1.5 years. Clinically, chronic persistent lip swelling involving upper lip and gingival enlargements were present along with systemic manifestations. Patient counseling with combination therapy showed excellent response with no signs of relapse at 6 months of follow-up. Conclusion: Cheilitis granulomatosa is a unique rare inflammatory disorder with many possible contributory factors and exact etiology is poorly understood. Clinical significance: This case report draws attention to constant exposure to an allergen that could be one of the possible etiological factors to CG secondary to pica eating disorder. Though it is a rare disease, research on the pathogenesis of CG and clinical trials evaluating the efficacy of the various treatments are needed to enhance our understanding of this disease, to improve the quality of life, as patients are very disturbed by the unsightly and distressing nature of macrocheilitis. How to cite this article: Dayanarayana U, Shastry SP, Kempegowda RT, et al. Management of Cheilitis Granulomatosa with Pica Disorder: A Rare Case Report. Int J Clin Pediatr Dent 2024;17(4):483-489.

Melkersson-Rosenthal syndrome: A case report.

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.

Intralesional Injections of a TNF-α Inhibitor to Treat Orofacial Granulomatosis.

We present the first documented case of successful treatment of orofacial granulomatosis by intralesional injections of a tumor necrosis factor α inhibitor to the lip. Our patient had rapid symptomatic improvement after 3 injections, and near resolution within 4 months of anti-tumor necrosis factor α therapy.

Orofacial granulomatosis as a manifestation of sarcoidosis: A rare case report.

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.

Patient with Crohn's Disease Presents for Pain in Relation to Maxillary Teeth.

Crohn's disease has been associated with poor oral health and oral health-related quality of life. Myriad-specific and nonspecific oral lesions have been associated with Crohn's disease. Oral lesions in patients with Crohn's disease may be a source of referred pain, especially if mucosal ulcerations or orofacial granulomatosis are involved. The dental provider can play an important role in evaluating for and/or managing oral lesions in patients with Crohn's disease and thereby improving patients' oral health and quality of life.

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.

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.

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.

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.

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

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.

Case report: Langerhans cell histiocytosis of the temporal bone in children: Challenging diagnosis of a rare disease with some pitfalls.

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.

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 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.

Erythematous plaques of the vulvo-perineal region.

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.

Orofacial granulomatosis: A case report and a proposal of a diagnostic algorithm for oral granulomatous lesions.

Orofacial granulomatosis (OFG) is an uncommon chronic inflammatory disorder with multifactorial etiology and pathogenesis affecting the orofacial region which appears usually as a persistent and/or recurrent swelling that may involve one or both the lips and/or recurrent ulcers along with other orofacial features. The diagnosis of OFG is challenging and it should be ruled out from other conditions characterized by granulomatous inflammation. This article describes a case of a 30-year-old female patient with upper lip swelling as the main manifestation without any systemic involvement and we have also laid down a proposal of a diagnostic algorithm for differential diagnoses and treatment modalities for OFG which could be helpful for the clinician and dentists alike to rule out other granulomatous disorders thus in effective management of OFG.

A Case of Melkersson-Rosenthal Syndrome Treated With 5-FU.

The rarity of Melkersson-Rosenthal syndrome, or orofacial granulomatosis, can present with persistent midface bogginess. The management for previous reported cases has included corticosteroid injections, antihistamines, and antibiotics. In the current reported case, the patient was treated with 5-fluorouracil and has been responding positively. Additionally, the patient has not shown signs of steroid atrophy.

An interesting case of lip swelling!

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.

Association between cheilitis granulomatosa and odontogenic infections: A case-control study.

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.

A case of orofacial granulomatosis evolving into Melkersson Rosenthal syndrome in a child treated with a steroid free regimen of Clofazimine and Minocycline.

Orofacial granulomatosis (OFG) is a rare disorder with varied etiological, immunological and infectious mechanisms implicated and is believed to be a umbrella term which includes Melkersson Rosethal syndrome (MRS). We describe a 17 year old female who was diagnosed with OFG and was successfully treated with a combination of minocycline and clofazimine without oral steroids with significant improvement within 1 month of therapy.