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Objective: To summarize the clinical phenotype and genetic characteristics of deficiency in ELF4 gene X-linked (DEX). Methods: A case series study was conducted to retrospectively analyze the clinical data and genetic testing results of 2 cases of DEX treated at Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and the First Hospital of Jilin University from January 2023 to April 2024. And literature up to April 2024 was searched from the PubMed database, as well as CNKI and Wanfang databases, using keywords such as "ELF4 deficiency" "deficiency in ELF4, X-linked""ELF4 gene". The main clinical manifestations and genotypes of DEX were summarized. Results: Both patients were male, with onset ages of 3 months and 3 years, respectively. Both patients presented with recurrent oral ulcers and abdominal pain. And the laboratory examination showed a significant increase in inflammatory markers. Intestinal examinations showed multiple intestinal ulcers, and both patients developed intestinal fistulas. Whole exome sequencing found ELF4 c.799C>T, p Arg267Trp and ELF4 c. 248-7G>A, both maternal variants. Based on clinical and genetic results, DEX were diagnosed. In terms of treatment, both patients underwent surgical treatment during the acute phase of the disease and received anti-tumor necrosis factor α therapy, but recurrent gastrointestinal symptoms were still observed in Patient 1, while the clinical effect in Patient 2 was still acceptable. However, the inflammatory markers in both patients were not normal even after treatment. Literature review found 18 patients including 2 patients in this study, reported in 5 English articles and no Chinese reports. Thirteen patients had disease onset age before 5. The main clinical manifestations were fever (12/17), oral ulcers (14/18), abdominal pain (8/18), diarrhea (6/18), perianal ulcers (5/17), ileum ulcers (6/16), colon ulcers (7/16), skin involvement (7/17) and recurrent infections (7/18); laboratory examinations found increased erythrocyte sedimentation rate (13/15) as well as C-reactive protein (9/9), and anemia (13/15); in terms of immunological function, there is a decrease in natural killer cells (9/15) as well as a decrease in class switching memory B cells (8/9). The main types of gene variantions were missense variantions (6/18), nonsense variantions (4/18) or frameshift variantions (3/18). Conclusions: DEX should be considered when an early-onset male patient manifested with recurrent fever, oral ulcers or mucosal ulcers, with elevated inflammatory markers, with or without recurrent infection. It is recommended to perform lymphocyte subsets analysis, gastrointestinal endoscopy and genetic testing to support the diagnosis.

Background: The course of late-onset Behçet's disease (LBD) is unknown. This study aimed to determine the characteristics and systemic involvement of LBD., Methods: In this retrospective, cross-sectional, and comparative study, 700 patient files from 4100 patients diagnosed with Behçet's disease (BD) were selected and divided into three groups: LBD, adult BD (ABD), and juvenile BD (JBD). The age limits for LBD and JBD were determined to be 42 and 16, respectively. LBD patients were compared to ABD and JBD patients in terms of demographics, systemic involvement, and disease duration., Results: The LBD rate among BD patients was 4.7% (183/4,100). The time from initial symptom occurrence to the age at which the BD criteria were met was longer in LBD than in ABD or JBD. Except for genital ulcers, the frequencies of involvement in LBD were similar to those in ABD and JBD. The frequency of family history was significantly lower in LBD (12%) than in ABD (15.9%) or JBD (18.2%). The time from the initial symptom to oral aphthae, genital ulcers, and eye involvement was longer in LBD than in ABD or JBD. Furthermore, erythema nodosum was observed after a longer duration in LBD than in ABD., Conclusion: Considering that involvement occurs much later in LBD and there are no differences in the frequencies of involvement except for genital ulcers, LBD patients should be followed up as closely as ABD and JBD patients., (© 2024 the International Society of Dermatology.)

In this case series, we present four unique cases of Riga-Fede disease (RFD), a rare disorder characterized by mucosal trauma as a result of repetitive tongue protrusion against the incisors, leading to the development of a large oral mass/ulceration. Due to the rapid development and growth of these lesions mimicking malignancy, it is important for the general and pediatric otolaryngologist to correctly diagnose and treat this benign disorder. This series highlights the variable clinical presentations, along with comorbidities of RFD, as well as the importance of interdisciplinary care between the pediatric otolaryngologist and pediatric dentist in its management. Laryngoscope, 134:4803-4805, 2024., (© 2024 The Author(s). The Laryngoscope published by Wiley Periodicals LLC on behalf of The American Laryngological, Rhinological and Otological Society, Inc.)

Objective: Inflammatory bowel diseases (IBD), such as Crohn's disease (CD) and ulcerative colitis (UC), are chronic conditions primarily affecting the intestines. This study aims to identify the nature and frequency of oral symptoms and signs in patients with ulcerative colitis and to explore the connection between these complications and the activity of the disease., Methods: This descriptive-analytical study involved ulcerative colitis patients who visited the gastroenterology clinic at Shahid Sayad Shirazi Hospital in Gorgan, along with an equal number of healthy companions as a control group in 2019. A gastroenterology internist assessed the disease severity through clinical examination and a simple clinical disease activity index for colitis patients. The collected data were then analyzed statistically using SPSS version 19., Results: The average age of patients in the case group was 41.84 ± 11.66 years, while in the control group it was 40.43 ± 12.67 years. There was a significant correlation between difficulty swallowing, burning sensation in the mouth, vomiting, acidic taste, the presence of oral ulcers, and a coated tongue with the severity of disease activity (p < 0.05). However, no significant relationship was found between other variables and the severity of disease activity (p > 0.05)., Conclusion: The results of this study confirm that as the severity of ulcerative colitis increases, the occurrence of oral lesions also rises, particularly during severe disease activity. Among oral symptoms, dry mouth had the highest incidence, followed by bad breath and changes in taste. The most common oral lesions observed were a coated tongue, grooved tongue, and oral ulcers, respectively., (© 2024. The Author(s).)

Background: Oral complications such as parageusia, xerostomia, and oral ulcers are frequently observed in patients with COVID-19. The aim of this study was to understand the oral complications and their influencing factors during the two waves of the COVID-19 pandemic in China between December 2022 and May 2023., Methods: A total of 347 patients during the COVID-19 pandemic from December 2022 and January 2023 and 163 patients during the second COVID-19 pandemic were recruited through the Disease Prevention and Control Reporting System. Data on oral complications were collected via researcher-made questionnaires., Results: During both pandemic periods, more than 50% of the subjects developed oral complications. Specifically, the incidence of parageusia, pathological tongue coating, and xerostomia all exceeded 20%, while the incidence of oral ulcers surpassed 7%. Patients with allotriosmia and xerostomia had a significantly higher likelihood of developing other oral complications. Hospitalized patients requiring supplemental oxygen had a significantly higher incidence of oral complications., Conclusions: The results of our study underline that oral complications during the COVID-19 pandemic are prevalent, and their incidence has not decreased during the subsequent pandemic. In the context of the ongoing global spread of COVID-19, this study lays the foundation for physicians in recognizing and managing oral complications, which will improve the oral health of communities globally., (© 2024. The Author(s).)

Traumatic ulcerative granuloma with stromal eosinophilia (TUGSE) is a reactive and chronic ulcerative lesion that is most frequently found on the tongue. It appears as a large yellowish ulcer with elevated margins and central induration. TUGSEs exhibit a characteristic pattern of regression often spontaneously, following incisional biopsy, or after removal of the potential traumatic trigger. Herein, we present an unusual case of a TUGSE on the anterior maxillary gingiva of a 70-year-old male that regressed following incisional biopsy. Histopathologic evaluation revealed fragments of squamous mucosa and submucosal tissue with large atypical cells mixed with small lymphocytes, abundant eosinophils, and focal clusters of plasma cells. Immunohistochemistry was performed and the large, atypical cells were positive for CD3, CD4, CD5, and CD30 and negative for CD1a, CD8, CD20, CD56, CD117, ALK1, Langerin, and EBER ISH. To our knowledge, this is the first case of TUGSE reported in the anterior maxilla., (Copyright © 2024 American Association of Oral and Maxillofacial Surgeons. Published by Elsevier Inc. All rights reserved.)

Traumatic Ulcerative Granuloma with Stromal Eosinophilia (TUGSE)/Riga Fede disease is a rare mucosal and submucosal benign reactive inflammatory process, usually involving the tongue. Trauma is believed to be a major factor amongst the multiple pathogenic mechanisms that have been hypothesized in TUGSE. The lesion presents as an isolated indurated or even ulcerated mass, which may mimic, clinically a squamous cell carcinoma (SCC). We herein report a case of TUGSE in a 63-year-old male referred by his treating physician for high suspicion of tongue malignancy. Histopathological examination confirmed the diagnosis of TUGSE, without any evidence of a neoplasic, infectious or hematologic process. TUGSE occurs in patients with an age range of 41-60 years. Sufficiently deep biopsies with comprehensive immunohistochemical and molecular analyses are mandatory to confirm the benign nature of the lesion and to, definitely, rule out malignancy. This report highlights the need for adequate histological differential diagnosis to avoid inappropriate heavy treatments in a benign condition., Competing Interests: Declaration of Competing Interest The authors declare that they have no conflict of interest regarding the publication of this article., (Copyright © 2023 Elsevier Masson SAS. All rights reserved.)

Background: Oral ulcerative mucositis (OUM) is common in patients with cancer, particularly in those undergoing chemoradiation therapy. The effective management of OUM is crucial for continuous cancer care and patient well-being. Recent studies have advanced our understanding of the causes, leading to clinical trials toward novel treatments. This review focuses on the contemporary therapeutic landscape, and provides the latest insights into the mechanisms of mucosal healing and pain., Highlights: Management strategies for OUM in patients with cancer include maintaining good oral hygiene, reducing mucosal irritation against radiation, and using various topical analgesic treatments, including herbal medicines. However, the current management practices have limitations that necessitate the development of more efficacious and novel treatments. Molecular research on transient receptor potential (TRP) channels in the oral mucosa is crucial for understanding the mechanisms of wound healing and pain in patients with OUM. Targeting TRPV3 and TRPV4 can enhance wound healing through re-epithelialization. The suppression of TRPV1, TRPA1, and TRPV4 may be effective in alleviating OUM-induced pain., Conclusion: Research advancements have improved our understanding and potentially led to novel treatments that offer symptomatic relief. This progress highlights the importance of collaborations between clinical researchers and scientists in the development of innovative therapies., (Copyright © 2024. Published by Elsevier B.V.)

Objective: This study aimed to assess the prevalence and types of oral adverse events following immunization (AEFIs) in people who received at least one dose of any type of vaccine., Materials and Methods: We conducted a bibliographic search about oral AEFIs in MEDLINE, Embase, PubMed, and Ovid from database inception to November 07, 2022. Risk of bias was assessed using the MURAD or the Quality In Prognosis Studies tools. Random-effects proportional meta-analysis was applied., Results: A total of 119 studies involving 343 people were eligible. These reported AEFIs occurred following administration of the coronavirus disease 2019 vaccine, anti-influenza vaccine, hepatitis B vaccine, and anti-smallpox vaccine. The most common to be affected in vaccinated people were buccal mucosa (63.1%; 95% confidence interval (CI) 33.4-88.2) and lips (55.7%; 95% CI, 41.1-69.8). The most prevalent oral AEFIs were ulceration (55.2%; 95% CI 24.4-84.0), swelling (65.2%; 95% CI 34.9-89.8), and burning sensation (18.3%; 95% CI 7.9-31.8)., Conclusions: The mechanisms underlying oral AEFIs should be further investigated to promptly recognize oral manifestations and provide optimal management for people undergoing vaccination., (© 2024 Wiley Periodicals LLC.)

Competing Interests: Potential conflicts of interest . The authors: No reported conflicts of interest. All authors have submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest. Conflicts that the editors consider relevant to the content of the manuscript have been disclosed.

Background: Paracoccidioidomycosis (PCM) is the leading cause of death among systemic mycoses in Brazil. On the other hand, oral squamous cell carcinoma (OSCC) is the most prevalent malignant neoplasm of the mouth. Both lesions rarely affect the tongue dorsum and may share similar clinical characteristics. This study aimed to retrieve cases of single oral ulcers diagnosed as PCM or OSCC., Material and Methods: A cross-sectional retrospective study was conducted. All patients who had a single ulcer on dorsum of the tongue and confirmed diagnosis of PCM or OSCC were evaluated., Results: A total of 9 patients (5 women and 4 men) were evaluated, 5 patients had OSCCs (mean age = 69,8 years old), and 4 patients PCM (mean age = 51 years old). Most of the lesions were infiltrated and indurated in the palpation exam. Duration ranged from 1 to 12 months (mean time of 5.2 months and 4.7 months for OSCC and PCM, respectively). OSCC was the main clinical diagnosis hypothesis., Conclusions: Although uncommon, PCM and OSCC should be considered as a diferential diagnosis hypothesis in infiltrated ulcers on the tongue dorsum. Iincisional biopsy is mandatory to confirm the diagnosis and indicate the appropriate treatment.

Abstract: Chronic nonhealing ulcers of the oral mucosa and lateral tongue, in particular, can transform into invasive oral squamous cell carcinoma (OSCC). Sometimes these ulcers do not heal even after the removal of the etiological agent that actually initiated these lesions, something similar to what happens in "neoplasia." Numerous factors have been postulated in the literature; however, the exact mechanism remains unclear. We hereby would suggest few plausible factors that could be considered for future studies to shed light on some untapped territories in the pathogenesis of OSCC arising from chronic nonhealing traumatic ulcers in purview of chromoanagenesis and the concepts of "quantum entanglement and coherence.", (Copyright © 2024 Copyright: © 2024 Indian Journal of Pathology and Microbiology.)

Objective: Opiorphin is a pentapeptide secreted in saliva and has a strong analgesic effect. Salivary opiorphin has been shown to increase in orofacial pain and may act as a pain reliever in pain caused by denture-related ulcers. The current study aimed to evaluate the salivary opiorphin levels in traumatic ulcers caused by ill-fitting dentures and demonstrate whether there is any correlation between trauma-related pain levels and salivary opiorphin levels., Materials and Methods: Twenty-two individuals with new full dentures and a complaint of pain due to ill-fitting were included in this study. Patients were asked to rate their level of pain on a visual analog scale (VAS). Then, saliva specimens were collected at the first visit with the complaint of pain and 7 days following the denture adjustment., Results: The average saliva opiorphin level before and after denture adjustment were 19.29 ± 5.44 and 15.78 ± 3.95 ng/mL, respectively. A dependent (paired) t-test determined that the mean salivary opiorphin level differed statistically significantly before and after the adjustment of the dentures., Conclusions: The findings show that salivary opiorphin levels increase in pain associated with denture-related traumatic ulcers. Adjusting the dentures resulted in pain relief and a statistically significant reduction in opiorphin levels., (© 2023 Wiley Periodicals LLC.)

Objective: Photobiomodulation therapy is recommended by multiple international societies for managing oral mucositis. These recommendations are based on extensive evidence. However, the search for an optimal photobiomodulation protocol continues. This mapping review focuses on a novel aspect of photobiomodulation therapy which is the immediate effect on pain levels associated with oral ulcerative conditions., Data Sources: This literature review systematically compiles and evaluates the evidence about oral mucositis, alongside other oral ulcerative conditions, as the protocols that achieved pain relief for these oral conditions may have potential applicability to oral mucositis management. The scientific database used was PubMed., Conclusion: Whereas most of the randomized controlled trials about photobiomodulation therapy for oral mucositis and other ulcerative oral diseases reported delayed pain relief, certain photobiomodulation therapy protocols reported immediate pain relief. The results of this review highlight the concept of preemptive photobiomodulation therapy, in which photobiomodulation therapy is delivered early in the development of oral mucositis throughout the oncotherapy and may achieve immediate pain relief consistently in most of the patients and close to a negligible pain level. Photobiomodulation therapy, as a powerful nonpharmacologic tool for immediate pain relief, has a great beneficial value in patients suffering from oral mucositis and other painful oral ulcerative diseases such as recurrent aphthous stomatitis and chronic graft-versus-host disease.

Competing Interests: Competing interests: The BMJ has judged that there are no disqualifying financial ties to commercial companies. The authors declare the following other interests: none.

Background: Oral traumatic ulcerative lesions (OTUL) are commonly encountered in clinical practice, yet there is limited research on their clinical characteristics and traumatic etiological factors. This retrospective study aimed to analyze the age, gender, clinical characteristics, and traumatic etiological factors in a large cohort of patients with OTUL and provide valuable insights for dental clinicians to optimize patient care and prevention strategies., Methods: A total of 1543 patients with OTUL were enrolled in this study. Age, gender, medical history, clinical characteristics and traumatic etiological factors were collected and analyzed. Logistic regression analysis was performed to determine the significance of age and gender as factors related to OTUL., Results: The study revealed significant variations in clinical characteristics and traumatic etiological factors among different age groups and between genders. Logistic regression analysis demonstrated that both age and gender were significant factors related to OTUL., Conclusion: The clinical characteristics of OTUL and traumatic etiological factors appear to be significantly different according to age and gender. More targeted prevention strategies should be implemented for all age and gender groups., (© 2024. The Author(s).)

Background: The aim of the study was to analyse the effects of Treatment Response with oral ulcers on oral health related quality of life in Behçet's syndrome (BS)., Material and Methods: In the cross-sectional study, 339 BS patients (F/M: 179/160, mean age: 36,13±9,81 years) were included. Data were collected by clinical examinations and patient reported outcome measures (PROMs) regarding Oral Health Impact Profile-14 (OHIP-14) questionnaire and self-reported Treatment Responses coded by a 5-point Likert-type scale (1: symptoms were cured- 5: symptoms were worsened). Moderated Mediation analysis (MA) was used to understand how oral ulcer activity (independent variable; X) influenced OHIP-14 score (outcome variables, Y) through self-reported Treatment Response (M1) and age (M2) as possible mediator variables (M) and disease course (mucocutaneous and musculuskeletal involvement vs. major organ involvement) as a possible moderator variable (W) on these relationships., Results: In Moderated MA, OHIP-14 score (Y) was mediated by the presence of oral ulcer (X) (p=0.0000), the negative Treatment Response (M1) (p=0.0001) and being young (M2) (p=0.0053) with mucocutaneous involvement (W)(p=0.0039)., Conclusions: Self-reported Treatment Response as an underestimated issue has a Mediator role in relation to oral ulceration on oral health related quality of life in the framework of patient empowerment strategies. Therefore, study results give clues to assist physicians and dentists for better understanding of patients' perspective.

Competing Interests: Competing interests: None declared.

Background: Riga-Fede disease (RFD) presents as oral mucosal ulceration in anatomical juxtaposition to sharp dental surfaces., Case Presentation: A 7-month-old healthy male presented to the pediatric dentist for an emergency appointment. His mother was concerned about episodic, recurrent, and excessive bleeding from his mouth for three weeks. She reported that her son would often wake up in "a pool of blood". The pediatrician assessed the patient when oral bleeding first started and prescribed magic mouthwash. This did not resolve the problem. The mother then took the child to an urgent care clinic, where she was referred to a pediatric dentist. The pediatric dentist noted a sublingual traumatic, granulomatous ulcer on the ventral surface of the tongue, extending up to the tip of the tongue. The mandibular primary central incisors had recently erupted. The pediatric dentist established a clinical diagnosis of RFD. Incisal edges were smoothened with dental polishing tips on a slow-speed handpiece and topical fluoride varnish was applied to the teeth. The size of the ulceration decreased over two weeks. However, in three weeks, there was another bleeding episode. The pediatric dentist smoothened the incisal edges again, followed by a fluoride varnish application. Eight weeks after the initial dental intervention, the lesion resolved completely without further bleeding., Discussion: The details of this case report highlight the maternal concerns, interprofessional educational touchpoints, scope for interprofessional collaborative care, and noninvasive management of Riga-Fede disease., Conclusion: In neurotypical patients, conservative dental management by smoothening sharp dental surfaces can resolve RFD. RFD should be differentiated from viral illnesses which present with oral ulcerations., (Copyright© Bentham Science Publishers; For any queries, please email at epub@benthamscience.net.)

Ulceration is probably the oral mucosal condition seen most frequently by general dental practitioners. It is almost always painful and therefore sufferers are prompt to seek advice. An important exception to this generalisation is the occurrence of oral squamous cell carcinoma, which is often painless in its early stages. Definitive diagnosis, which requires mucosal biopsy, is mandatory for any persistent area of oral ulceration., (© 2023. Springer Nature Switzerland AG 2019. Republished.)

Pemphigus vulgaris (PV) is a chronic autoimmune mucocutaneous blistering disease. Autoantibodies are directed against desmogleins, leading to the formation of intraepithelial bullae. PV, as with other autoimmune mucocutaneous disorders of the oral cavity, presents diagnostic and therapeutic challenges. Approximately 50-70% of cases present first with oral lesions. The lesions commonly start as vesicles or bullae that rapidly rupture, leading to erosions and ulcerations. The palatal, gingival, buccal, and labial mucosa are the most commonly affected sites. Oral PV can mimic several other diseases that cause mucosal erosions and/or ulcerations, including erythema multiforme (EM). EM is an acute, immune-mediated, self-limited hypersensitivity condition primarily associated with herpes simplex infection. Oral lesions can be variable, but a very characteristic presentation with labial hemorrhagic erosions, ulcerations and crusting is commonly seen. In this case series, we present six cases of PV: one male patient and five female patients whose ages ranged from 34 to 65 years old. All patients presented with hemorrhage and crusting of the lips in addition to multiple intraoral erosions and ulcerations. Three patients presented with oral and skin lesions. All patients underwent biopsies, and a diagnosis of PV was confirmed. All patients were treated with steroids (topical and systemic) and variable steroid-sparing agents. This case series emphasizes that oral PV may be misdiagnosed as EM in a subgroup of patients who present with persistent lip hemorrhage and crusting. Therefore, a comprehensive history, clinical examination and incisional biopsies should be considered in such patients., (© 2023. The Author(s).)

Ulceration is probably the oral mucosal condition seen most frequently by general dental practitioners. It is almost always painful and therefore sufferers are prompt to seek advice. An important exception to this generalisation is the occurrence of oral squamous cell carcinoma, which is often painless in its early stages. Definitive diagnosis, which requires mucosal biopsy, is mandatory for any persistent area of oral ulceration., (© 2023. Springer Nature Switzerland AG 2019. Republished.)

Neutropenic ulcerations are characterized by mucosal ulcerations which occur in the presence of neutropenia, suggesting a direct link between neutropenia and mucosal ulceration. An oral ulcer can be labeled as "neutropenic" only if the patients have primary (typically congenital) or secondary neutropenia, and neutropenia is the sole causative factor. Oral mucosal ulcers observed in patients undergoing oncologic therapy may also be termed as "neutropenic ulcers", but the pathogenesis of these oral ulcers more likely involves mucosal events related to trauma, microbial factors, and direct cytotoxicity. In cancer patients, the early appearance of oral ulcers is often attributed to oral mucositis which is a condition primarily caused by the direct mucosal cytotoxicity of chemotherapeutic agents and radiation therapy. Oral ulcers that develop later during or after active cancer therapy may result from intraoral trauma and typically manifest on non-keratinized areas of the oral mucosa which are more susceptible to mucosal damage. In patients undergoing chemotherapy, factors such as disturbances in mucosal barrier function as well as bone marrow suppression lead to reduced neutrophil count and function, and can contribute to the development of oral ulcers. While the etiology of oral ulcers in cancer therapy receiving patients can vary, it is important to emphasize that the host's response plays a crucial role in the progression and repair process of these lesions. This narrative review presents the etiopathogenesis, clinical presentation, and potential management approaches for oral ulcerations in neutropenic patients, with a particular focus on clarifying the usage of the term "neutropenic ulcer" since this term lacks diagnostic specificity and can be misleading in clinical practice regarding the underlying causes and treatment strategies., (© 2023. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objectives: To define the clinical characteristics of oral ulceration (OU) in Behçet's disease (BD), to allow differentiation from other causes of OU, including aphthous ulcers, by an International Delphi consultation. To develop a clinical guideline on how to recognise BD ulcers., Methods: Round 1. 40 clinical images of OU in BD, recurrent aphthous stomatitis (RAS), inflammatory bowel disease (IBD) and mucous membrane pemphigoid (MMP) were shown. Participants answered, independently, which images would be consistent with a BD ulcer. Round 2. The results from marking independently were shown. The panel remarked the questions through iteration process. The images not agreed to be a possible BD ulcer were discarded. Round 3. 10 clinical descriptors that may define BD ulcers were suggested. Participants ranked the level of importance for each descriptor on each image presented. Round 4. Participants re-ranked their level of agreement for each descriptor through iteration process. Whether the clinical pictures would be different from RAS was also explored. A final agreement was reached., Results: This study has shown clear differentiation between BD, IBD and MMP ulcers when defining them by phenotype through clinical images only. On the other hand, no differentiation between RAS and BD ulcers was found. The most important clinical descriptors that define BD ulcers have been agreed., Conclusions: New clinical guidance for Health Care Professionals (HCP) on how to recognise a BD ulcer has been proposed. This should elucidate an earlier diagnosis, quicker access to treatment and control of the disease enhancing patient's quality of life.

Objectives: The study aimed to identify the interactions among treatment protocols and oral ulcer activity related factors in patients with Behçet's syndrome (BS) using the Classification and Regression Tree (CART) algorithm., Methods: In this cross-sectional study, 979 patients with BS were included from16 centres in Turkey, Jordan, Brazil and the United Kingdom. In the CART algorithm, activities of oral ulcer (active vs. inactive), genital ulcer (active vs. inactive), cutaneous involvement (active vs. inactive), musculoskeletal involvement (active vs. inactive), gender (male vs. female), disease severity (mucocutaneous and musculoskeletal involvement vs. major organ involvement), smoking habits (current smoker vs. non-smoker), tooth brushing habits (irregular vs. regular), were input variables. The treatment protocols regarding immunosuppressive (IS) or non-IS medications were the target variable used to split from parent nodes to purer child nodes in the study., Results: In mucocutaneous and musculoskeletal involvement (n=538), the ratio of IS use was higher in patients with irregular toothbrushing (ITB) habits (27.1%) than in patients with regular toothbrushing (RTB) habits (14.2%) in oral ulcer activity. In major organ involvement (n=441), male patients with ITB habits were more likely treated with IS medications compared to those with RTB habits (91.6% vs. 77.6%, respectively)., Conclusions: Male BS patients on IS who have major organ involvement and oral ulcer activity with mucocutaneous and musculoskeletal involvement have irregular toothbrushing habits. Improved oral hygiene practices should be considered to be an integral part for implementing patient empowerment strategies for BS.

Objective: To investigate the risk factors of oral ulcers and bloodstream infection in patients with hematopoietic stem cell transplantation., Methods: The clinical data of 401 hematopoietic stem cell transplant patients in the First Affiliated Hospital of Zhengzhou University from January 2020 to December 2021 were retrospective analyzed, and the risk factors of oral ulcers and bloodstream infection statistical and analyzed., Results: Among the 401 patients, the incidence of oral ulcers was 61.3% (246/401), and the incidence of bloodstream infection was 9.0% (36/401). A total of 40 strains of pathogenic bacteria were isolated from 36 patients, including 26 strains of Gram negative strains (65%), 13 strains of Gram positive strains (32.5%), and 1 strain of fungi (2.5%). Single-factor analysis showed that oral hygiene was associated with the occurrence of bloodstream infection, and the Multi-factor analysis showed that age ≥14 years old, disease diagnosis of leukemia, and allogeneic hematopoietic stem cell transplantation were risk factors for oral ulcers., Conclusion: The incidence of oral ulcers in patients with hematopoietic stem cell transplantation is high. The age ≥14 years, disease diagnosis of leukemia, and allogeneic hematopoietic stem cell transplantation were risk factors for oral ulcers in patients, and oral hygiene was associated with the occurrence of bloodstream infection.

Ulceration is a common presenting sign of a wide spectrum of diseases of the oral cavity involving many etiologic factors, such as trauma, infection, neoplasms, medication, and immune related disorders, ranging from self-limited lesions to life-threatening diseases. In most cases, a proper diagnosis can be established based on medical history and clinical features only. Early diagnosis is important as oral ulcerations might be a manifestation of a systemic disease or sometimes even due to a malignant process.

Objectives: To describe the long-term clinical course of each manifestation of Behçet's disease (BD) and clarify factors involved in oral ulcer (OU) remission using clinical information of BD patients., Methods: We retrospectively studied 155 BD patients visiting our hospital (1989-2020). We defined remission criteria for each manifestation and examined long-term clinical changes. Classification and regression trees and multivariable analyses were performed to investigate OU prognostic factors; hazard ratios were used to assign scores to prognostic factors deemed significant [OU prognosis score (OuP score)]. Risk stratification was examined by dividing the OuP scores into four stages., Results: OUs appeared earliest, with the slowest decline in prevalence observed post-BD diagnosis. OU presence was the most common factor inhibiting complete remission. Young age at OU onset, never smoker, presence of genital ulcers, positive pathergy test, no usage of tumour necrosis factor inhibitors or of immunosuppressants, and long-term non-treatment or symptomatic treatment for OUs were poor OU prognostic factors. Based on multivariable analysis, the area under the curve of the OuP score to predict OU prognosis was 0.678., Conclusions: Remission criteria for each symptom clarified that OU had the greatest impact on complete BD remission. Faster OU remission was associated with earlier OU therapeutic intervention other than symptomatic treatment., (© Japan College of Rheumatology 2022. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)

The aim of this article is to present the case of acute febrile neutrophilic dermatosis (Sweet syndrome-SS) after Ad26.COV2.S vaccination against SARS-CoV-2. To the best of our knowledge, this is the second case of SS provoked by this specific vaccine. What is more, the mildly symptomatic beginning of the disease, later followed by typical SS manifestation with a variety of symptoms including nodular erythema of the feet and oral ulcerations, made it very challenging to establish the diagnosis. The article focuses on the current literature on the acute febrile neutrophilic dermatosis, along with the coexistence with other neutrophilic dermatoses and anti-SARS-CoV-2 vaccinations as provoking factors. It emphasizes the necessity for sharing the knowledge and experience on the subject of SS's clinical manifestations and underlying causes to facilitate prompt diagnosis and introduction of appropriate treatment., (© 2022 The Authors. Dermatologic Therapy published by Wiley Periodicals LLC.)

Eosinophilic ulcer of the oral mucosa is a benign, reactive, self-limited lesion. Its pathogenesis is unclear, and it clinically manifests as a punched-out ulcer with surrounding indurated border, raising suspicion for a possible malignancy. On histology, an acute and chronic dense inflammatory infiltrate can be appreciated, which can extend to the deep underlying tissue (including skeletal muscle). Eosinophils are especially conspicuous on hematoxylin and eosin staining and might play an important role in the development of the ulcer. Removal of possible triggers followed by monitoring and possible biopsy are among the initial recommendations. Most lesions heal spontaneously without the need for intervention. This review aims at assisting dermatologists to easily recognize this entity, avoiding unnecessary interventions and apprehension., (© 2021 the International Society of Dermatology.)

Rationale: Behçet's disease (BD) is an autoimmune disease presented with recurrent oral aphthous ulcers, genital ulcers, uveitis and skin lesions. The cutaneous manifestations are often refractory and more resistant to conventional therapy. This report provides a new treatment of low-dose interleukin-2 (IL-2) with refractory BD patient., Patient Concerns: A 37-year-old female patient experienced oral ulcer and erythema nodosum on the right leg for over 12 months and resisted to Methylprednisolone and Thalidomide., Diagnoses: The patient suffered from recurrent painful oral ulceration and an erythema nodosum. Pathergy test is also positive. Thus, we diagnosed her as BD according to the International Criteria for Behçet's Disease (ICBD)., Interventions: The patient took Methylprednisolone 8 mg qd, Thalidomide 50 mg qn and Hydroxychloroquine 200 mg bid successively as treatment. However, the medicine didn't take effect. Finally, this patient was given low-dose IL-2 intramuscular injection qod for 3 months., Outcomes: Oral ulcers and the erythema disappeared and the patient has been symptom-free for 6 months., Lessons: low-dose IL-2 is a safe and effective treatment for refractory BD., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.)

Objectives: This study aimed to determine the clinical efficacy of apremilast for oral ulcers (OUs), extra-oral manifestations, and overall disease activity in patients with Behçet's disease (BD)., Methods: A systematic literature search was performed in PubMed, Embase, Cochrane Library, and Web of Science Core Collection. Studies assessing the treatment effects of apremilast in BD were included. The odds ratios (ORs) of being symptom-free for individual manifestations and mean difference (MD) of Behçet's Disease Current Activity Form (BDCAF) scores were calculated with 95% confidence intervals (CIs) at 12 and 24 weeks using a random-model meta-analysis., Results: Of 259 screened articles, eight were included. After 12 weeks of apremilast treatment the OR of symptom-free was as followings: OUs, 45.76 (95% CI, 13.23-158.31); genital ulcers, 4.56 (95% CI, 2.47-8.44); erythema nodosum, 3.59 (95% CI, 1.11-11.61); pseudofolliculitis, 2.81 (95% CI, 1.29-6.15); and arthritis, 3.55 (95% CI, 1.71-7.40). Furthermore, BDCAF scores at 12 weeks were significantly reduced (MD=-1.38; -1.78 to -0.99). However, the proportion of oral-ulcer-free patients increased at 24 weeks (OR = 14.88; 4.81 to 46.07)., Conclusions: The currently accumulated data indicate an improvement in mucocutaneous and articular symptoms by short-term apremilast treatment in patients with BD., (© Japan College of Rheumatology 2021. Published by Oxford University Press. All rights reserved. For permissions, please e-mail: journals.permissions@oup.com.)