Prospective Assessment of Glucocorticoid Toxicity in Rheumatology Practice: A Focus on the Glucocorticoid Toxicity Index.

OBJECTIVE: The glucocorticoid toxicity index (GTI) is developed to measure glucocorticoid (GC)-related morbidity over time. This study aimed to assess GC-toxicity in patients at a rheumatology outpatient clinic by using the GTI and to identify the factors that interfere with the GTI. METHODS: This prospective study included patients with inflammatory arthritis (IA), connective tissue disease, and vasculitis who were newly prescribed GC-treatment (GC-naive) or have been still on GC-treatment for ≤2 years (GC-experienced). Patient demographics and disease characteristics, aggregate improvement score (GTI-AIS), cumulative worsening score (GTI-CWS), and cumulative GC-doses were recorded at baseline, 3rd month, and 6th month. Generalized estimating equations (GEE) were used to evaluate the GTI scores and associated factors including cumulative GC-doses. RESULTS: The study included 156 (48.7% GC-naive) patients with a mean age of 49.1 ± 17.1 years. More than half of the patients in both groups had a diagnosis of vasculitis. A higher cumulative GC-dose was found to be associated with higher GTI-scores in both groups (p< 0.001). In the GC-naive group, patients with vasculitis showed higher GTI-scores than IA patients (p< 0.001); there was also a significant increase in the GTI-CWS at the 6th month compared with the 3rd month. In the GC-experienced group, GTI-AIS and GTI-CWS were significantly different at 3rd and 6th month (p< 0.05). CONCLUSION: It was shown that GTI scores were associated with cumulative GC-doses and vasculitis patients in the GC-naive patients had higher GTI scores than inflammatory arthritis. The GTI allows individualized assessment and management of adverse effects experienced by patients as a result of GC treatment.

'Eczematous' dermatitis of the nipple: clinical and histopathological differential diagnosis of Paget disease.

The nipple can be affected by many malignant and benign entities. A wide variety of diseases including Paget disease, atopic dermatitis and nipple candidiasis can cause eczema-like changes in the nipple. In cases of diagnostic uncertainty, tissue sampling may be indicated. A true eczematous lesion, such as atopic dermatitis, typically shows a spongiotic dermatitis pattern. Paget disease, on the other hand, presents with infiltration of the nipple epidermis by neoplastic cells. The presence of atypical cells scattered in the epidermis in a pagetoid pattern opens up a histopathological differential diagnosis encompassing squamous cell carcinoma in situ and malignant melanoma, among others. Immunohistochemistry is commonly used to render a diagnosis. The objective of this article is to discuss Paget disease and highlight relevant clinical and histopathological differential diagnoses.

Validity and intra- and inter-rater reliability of the Tinetti performance-oriented mobility assessment balance subscale using different tele-assessment methods in patients with chronic stroke.

INTRODUCTION: Balance assessments are an important component of rehabilitation. Considering the increasing use of telemedicine to meet rehabilitation needs, it is important to examine the feasibility of such assessments. This study aimed to examine the reliability and validity of the Tinetti Performance-Oriented Mobility Assessment Balance Subscale (POMA-B) when applied via synchronous and asynchronous tele-assessment. METHODS: Twenty-five patients with chronic stroke were included in the study. The first physiotherapist assessed the patients on the first day in a face-to-face clinical setting. Synchronous or asynchronous tele-assessment was applied the next day. The assessments were performed in the same time zone, with an interval of one day. The synchronous tele-assessment was done online in real time by the first and second physiotherapists. A reference assessment video was sent to the patients for asynchronous tele-assessment. They were asked to make a video recording while performing the evaluation activities according to the reference video. Then the first and second physiotherapists assessed these video recordings separately. All the tests were repeated 10 days later to determine the intra-rater reliability of the tele-assessment methods. RESULTS: The intra-class correlation coefficients ranged from 0.96 to 0.98 for inter-rater reliability and from 0.97 to 0.98 for intra-rater reliability for both tele-assessment methods. Both asynchronous and asynchronous tele-assessment methods were medium correlated with the face-to-face versions. CONCLUSIONS: We demonstrated the validity and reliability of the POMA-B in chronic stroke patients with different tele-assessment methods, typically using the internet and available devices.

Biomarker detection using corrected degree of domesticity in hybrid social network feature selection for improving classifier performance.

BACKGROUND: Dimension reduction, especially feature selection, is an important step in improving classification performance for high-dimensional data. Particularly in cancer research, when reducing the number of features, i.e., genes, it is important to select the most informative features/potential biomarkers that could affect the diagnostic accuracy. Therefore, researchers continuously try to explore more efficient ways to reduce the large number of features/genes to a small but informative subset before the classification task. Hybrid methods have been extensively investigated for this purpose, and research to find the optimal approach is ongoing. Social network analysis is used as a part of a hybrid method, although there are several issues that have arisen when using social network tools, such as using a single environment for computing, constructing an adjacency matrix or computing network measures. Therefore, in our study, we apply a hybrid feature selection method consisting of several machine learning algorithms in addition to social network analysis with our proposed network metric, called the corrected degree of domesticity, in a single environment, R, to improve the support vector machine classifier's performance. In addition, we evaluate and compare the performances of several combinations used in the different steps of the method with a simulation experiment. RESULTS: The proposed method improves the classifier's performance compared to using the whole feature set in all the cases we investigate. Additionally, in terms of the area under the receiver operating characteristic (ROC) curve, our approach improves classification performance compared to several approaches in the literature. CONCLUSION: When using the corrected degree of domesticity as a network degree centrality measure, it is important to use our correction to compare nodes/features with no connection outside of their community since it provides a more accurate ranking among the features. Due to the nature of the hybrid method, which includes social network analysis, it is necessary to investigate possible combinations to provide an optimal solution for the microarray data used in the research.

Malignant solitary fibrous tumor of the urinary bladder progressing to widespread metastases and death: a rare case report and literature review.

Solitary fibrous tumor (SFT) of the urinary bladder has been rarely reported and malignant bladder SFT is even rarer. Here we present a case of an African-American male with SFT of the urinary bladder (intermediate risk) initially treated by cystoprostatectomy at the age of 59 years. Eight years later, he developed recurrence with widespread metastases to the liver, lungs, and abdominal cavity. He then received temozolomide and bevacizumab with good disease control. However, treatment was paused due to declining performance status. Follow-up at 1 year demonstrated growth of the metastatic lesions. Despite restarting therapy, the patient expired, 11 years after the original diagnosis. Autopsy was performed and revealed widespread metastases within the abdominal cavity (abdominal sarcomatosis) as well as liver, bilateral lung, and diaphragmatic involvement. The cause of death was determined to be metastatic SFT. A comprehensive literature review was performed. Although SFTs are commonly considered benign, a subset of SFTs of the urinary bladder behave aggressively. Risk assessment and proper follow-up for recurrence and metastasis is necessary. The patient was also found at autopsy to have two gastrointestinal stromal tumors (GISTs) in the stomach and near the gastroesophageal junction. To the best of our knowledge, this is the first reported case of a primary urinary bladder SFT resulting in death or having concurrent, multifocal GISTs, and only the second case of a bladder SFT that developed metastases after the initial diagnosis.

Fit of High-Translucency Zirconia Fixed Dental Prostheses Using Two Different Intraoral Scanners.

PURPOSE: To assess the marginal and internal fit of monolithic zirconia fixed dental prostheses (FDPs) comparing two different intraoral scanners (IOSs) and assessment methods. MATERIALS AND METHODS: A maxillary typodont with three prepared teeth allowed fabrication of five-unit high-translucency monolithic zirconia FDPs. The typodont was scanned with two IOSs: Cerec Omnicam and 3Shape TRIOS 3. In total, 30 FDPs were milled from presintered high-translucency zirconia blocks. Dual-scan and silicone replica assessments were used to determine marginal and internal gaps. RESULTS: A statistical significance was observed for marginal gaps (3Shape [74.27 ± 10.4 µm] and Cerec [86.45 ± 14.3 µm]) and for internal gaps (3Shape [126.12 ± 12.7 µm] and Cerec [112.38 ± 17.2 µm]). CONCLUSIONS: The outcomes of this study showed that the 3Shape TRIOS 3 produced a better overall marginal fit, while the Cerec Omnicam created a better internal fit. Additionally, a greater overall marginal and internal gap was noted with the silicone replica method over the dual-scan method.

Do cervical stabilization exercises change the effects of conventional exercises in patients with Parkinson's disease?

OBJECTIVES: The aim of this study was to examine whether cervical stabilization exercises (CSEs) change the effects of conventional exercises (CEs) in patients with PD. METHODS: Twenty-five patients with PD were randomized into two groups. While the experimental group (EG) received CSEs in addition to CEs, the control group (CG) received only CEs. Both programs lasted 8 weeks. Eighteen participants were able to complete the study. The outcomes were the changes in posture, cervical joint position sense (JPS), balance assessment, 10-m walking tests (10MWT), and the Timed Up & Go (TUG) test. RESULTS: At the end of the study, significant improvement was observed in both groups in terms of trunk rotation angle and pelvic asymmetry, the time parameter of TUG, and the 10MWT (p < 0.05). In the EG, greater improvement was detected in the Berg Balance Scale, static posturography, postural alignment, JPS, and the cadence parameter of TUG (p < 0.05). DISCUSSION: CEs and CSEs could improve walking speed and posture in patients with PD, but if CSEs are added to CEs, greater improvements could be achieved in JPS and postural control.(Clinical Trials ID: NCT03854747).

Early Postoperative Adjuvant Radiotherapy Versus Active Monitoring After Gross Total Resection for Atypical Meningiomas: Factors Associated with Early Recurrence.

AIM: To investigate the predictors of recurrence after gross total resection (GTR) that require early adjuvant radiotherapy upfront rather than at initial recurrence of atypical meningiomas (AMs). MATERIAL AND METHODS: A retrospective study of gross totally resected AMs was conducted in a tertiary care center within ten years. The clinical, radiological, and pathological parameters were analyzed statistically, and the factors associated with recurrence after GTR were determined with univariate analysis. RESULTS: Among 23 AMs with GTR, 34.8% showed recurrence in a median follow-up of 40 months after the surgery. Preoperative tumor volume, tumor location in the skull base or tentorium, and lack of progesterone expression were associated with the higher recurrence rate. AMs with a preoperative volume of 27.5 cm³ were the most significant risk factor for the recurrence (a 9.3-fold increase) than those with < 27.5 cm < sup > 3 < /sup > (66.7% vs. 14.3%, respectively). CONCLUSION: Patients diagnosed with larger AMs ( > 27.5 cm < sup > 3 < /sup > ) might have higher recurrence rates after GTR and, therefore, would benefit from early adjuvant radiotherapy without waiting for a recurrence. AMs located in the skull base or tentorium and AMs having no progesterone expression might also be potential predictors for recurrence.

TRPS1: A Marker of Follicular Differentiation.

The trichorhinophalangeal syndrome type 1 (TRPS1) immunohistochemical (IHC) stain has increased in use in recent years as a marker for breast carcinomas. The TRPS1 gene is involved in various tissues, including the growth and differentiation of hair follicles. This article seeks to evaluate the IHC expression of TRPS1 in cutaneous neoplasms with follicular differentiation, such as trichoblastoma (TB), trichoepithelioma (TE), and basal cell carcinoma (BCC). IHC studies were performed on 13 TBs, 15 TEs, and 15 BCCs with an antibody against TRPS1. The study found a variable staining expression of TRPS1 in the tumor nests of TB, TE, and BCC. BCCs were distinct in that none of the BCCs demonstrated intermediate or high positivity, while TBs and TEs showed intermediate-to-high positivity in 5/13 (38%) and 3/15 (20%) of cases, respectively. We observed a distinct staining pattern among the mesenchymal cells of TB and TE. We found that TRPS1 highlighted perifollicular mesenchymal cells adjacent to the nests of TB and TE tumor cells. This staining pattern was absent in BCCs, where only scattered stromal cells were positive for TRPS1. Papillary mesenchymal bodies were also highlighted by TRPS1 in TB and TE. TRPS1 stained various parts of the normal hair follicle, including the nuclei of cells in the germinal matrix, outer root sheaths, and hair papillae. TRPS1 may be a useful IHC marker for follicular differentiation.

Two distinct pathogenic pathways of digital papillary adenocarcinoma - BRAF mutation or low-risk HPV infection.

Digital papillary adenocarcinoma (DPA) is a rare neoplasm that can exhibit local recurrence and distant metastasis. We present a series of eight cases of DPA showing two distinct clinical presentations, morphologies, immunophenotypes, and molecular features. Four cases were characterized by painless, slow-growing nodules located on the digits. The lesions were small, well-defined, and confined in the dermis. Histopathologically, these tumors were composed of glandular structures lined by cuboidal epithelium with luminal papillary infoldings. Only rare mitotic figures and minimal squamoid differentiation were present, and cellular necrosis was absent. All four cases were positive for the BRAF V600E immunohistochemistry but negative for p16, low-risk and high-risk HPV in situ hybridization (ISH). In contrast, the remaining four cases were characterized by painful, rapidly growing masses on the digits. These four lesions were located in the deep dermis and consisted of a solid, tightly packed papillary architecture lined by atypical epithelioid cells with inconspicuous nucleoli. Cellular necrosis, numerous mitotic figures, and prominent squamoid differentiation were seen. All cases were negative for the BRAF V600E IHC. However, they showed strong, patchy to diffuse reactivity for p16 and were positive for low-risk HPV ISH and negative for high-risk HPV ISH. Our findings suggest that the current classification of DPA encompasses tumors that show two discrete pathogenic pathways - BRAF mutation or low-risk HPV infection. DPAs with low-risk HPV infection exhibit aggressive clinical features, high-grade morphology, marked squamoid differentiation, and wild-type BRAF. DPAs with BRAF V600E have less aggressive clinical features, low-grade morphologic findings, mild to absent squamoid differentiation, and negative HPV infection.

TRPS1 Is Differentially Expressed in a Variety of Malignant and Benign Cutaneous Sweat Gland Neoplasms.

Neoplasms of sweat glands and the breast may be morphologically and immunophenotypically similar. A recent study showed that TRPS1 staining is a highly sensitive and specific marker for breast carcinoma. In this study, we analyzed TRPS1 expression in a spectrum of cutaneous sweat gland tumors. We stained five microcystic adnexal carcinomas (MACs), three eccrine adenocarcinomas, two syringoid eccrine carcinomas, four hidradenocarcinomas, six porocarcinomas, one eccrine carcinoma-NOS, 11 hidradenomas, nine poromas, seven cylindromas, three spiradenomas, and 10 syringomas with TRPS1 antibodies. All of the MACs and syringomas were negative. Every cylindroma and two of the three spiradenomas demonstrated intense staining in cells lining the ductular spaces, with negative to relatively weak expression in surrounding cells. Of the 16 remaining malignant entities, 13 were intermediate to high positive, one was low positive, and two were negative. From the 20 hidradenomas and poromas, intermediate to high positivity was revealed in 14 cases, low positivity in three cases, and negative staining in three cases. Our study demonstrates a very high (86%) expression of TRPS1 in malignant and benign adnexal tumors that are mainly composed of islands or nodules with polygonal cells, e.g., hidradenomas. On the other hand, tumors with small ducts or strands of cells, such as MACs, appear to be completely negative. This differential staining among types of sweat gland tumors may represent either differential cells of origin or divergent differentiation and has the potential to be used as a diagnostic tool in the future.

FLI-1/Melan-A dual stain is an alternative to PRAME in differentiating metastatic melanoma from nodal nevus: A monocentric retrospective study.

Melanocytic nevi existing in lymph nodes create a diagnostic challenge by mimicking metastases. PReferentially expressed Antigen in MElanoma (PRAME) immunohistochemical (IHC) stain can differentiate one from another. FLI-1 IHC expression has been shown in malignant melanoma with variable sensitivity while melanocytic nevi were reported to be negative. We hypothesized that FLI-1/Melan-A dual IHC staining may be used in the distinction of metastatic melanoma from nodal nevi and can be an alternative and/or complementary to PRAME. In this study, we examined 13 lymph nodes with metastatic melanoma and 13 lymph nodes with benign deposits. We stained all of the lymph nodes with FLI-1, FLI-1/Melan-A dual, and PRAME IHC stains. In addition, we stained paired skin samples of the metastatic lymph nodes with FLI-1 and PRAME. In primary cutaneous melanomas, 11 of 13 were positive for FLI-1 and PRAME expression (85%). Malignant cells in 12 and 13 lymph nodes showed positive expression of PRAME and FLI-1, respectively. Only one case with a nevic cell deposit was weakly positive for FLI-1 and the remaining benign cases were negative for both FLI-1 and PRAME. Our results show that FLI-1/Melan-A dual stain is as sensitive and specific as PRAME in distinguishing lymph nodes with metastatic melanoma from nodal nevi. Further studies with larger case numbers are needed to support our significant results.

Effects of Two Exercise Regimes on Patients with Chiari Malformation Type 1: a Randomized Controlled Trial.

This study aims to measure the effects of two different exercise programs on neck pain, proprioception, balance, coordination, posture, and quality of life in patients with Chiari malformation (CM) type 1. Sixteen patients were randomized to two different exercise programs: a tailored exercise protocol for CM (TEP-CM) and cervical spinal stabilization exercises (CSSE). Both exercise programs were implemented by a physiotherapist 3 days a week for 6 weeks. The primary outcome was Neck Disability Index. Secondary outcomes were visual analogue scale for pain, joint position sense error measurement, Berg Balance Scale, Time Up and Go Test, International Coordination Ataxia Rating Scale, PostureScreen Mobile, and Short Form-36. Assessments were done immediately before and after the intervention programs. Both groups showed significant improvement in Neck Disability Index, and some secondary outcome measures (P < 0.05). However, there were no statistical differences in post-intervention changes between the groups (P > 0.05). This is the first study to examine the effects of different exercise programs on symptoms in patients with CM type 1. Our preliminary findings indicate that exercise programs can improve pain, balance, proprioception, posture, coordination, and quality of life in CM type 1. Therefore, exercise should be considered safe, beneficial, and low-cost treatment option for CM type 1 patients without surgical indications.

Claudin-4 Upregulation in Acantholytic and Autoimmune-Mediated Bullous Disorders.

Claudin-4 is a key component of tight junctions, which play an important role in the formation of the epidermal barrier by forming a circumferential network in the granular layer that serves as a gatekeeper of the paracellular pathway. The aim of this study is to illustrate claudin-4 immunohistochemical staining patterns of different blistering disorders. We collected 35 cases, including two Hailey-Hailey disease, one Darier disease, three Grover disease, one acantholytic acanthoma, two warty dyskeratoma, 11 pemphigus vulgaris (PV) including six mucosal PV, and two pemphigus foliaceus. For comparison, we included five cases of normal skin, five eczema, and three bullous pemphigoid cases. Claudin-4 demonstrated weak-to-moderate expression in keratinocytes located in the stratum granulosum, keratinocytes surrounding hair follicles, and adnexal glands. Further, claudin-4 exhibited moderate-to-strong membranous staining in disrupted keratinocytes surrounding and within the acantholytic and bullous areas in 16/22 of the acantholytic cases (not seen in the six cases of mucosal PV) and all three bullous pemphigoids. This finding suggests that claudin-4 is upregulated in these conditions, which may be a compensatory response to the disrupted barrier function. This finding could shed light on the molecular mechanisms underlying disrupted barrier function in blistering disorders, independent of the specific underlying disease mechanism.

Assessment of Mucosa-Associated Epithelial Chemokine, Thymus-Expressed Chemokine, Periostin and Zonulin Levels in Infants With Atopic Dermatitis.

Background: Atopic dermatitis (AD) is a chronic inflammatory skin disease. Skin and gut are the organs that first encounter antigens and environmental triggers. The mechanisms behind the relation between skin and gut immune responses in AD have not been identified yet. Aims and Objectives: To investigate mucosa-associated epithelial chemokine (MEC/CCL28), thymus-expressed chemokine (TECK/CCL25), periostin and zonulin levels in infants with AD. Materials and Methods: Children under one year old participated in the study. We used a propensity matching score. We included 39 infants who had active AD lesions at the time of evaluation. Serum MEC/CCL28, TECK/CCL25, periostin and zonulin levels were measured. Results: We examined age and sex matched 39 infants with AD and 39 healthy infants. Median value of zonulin was lower in infants with AD [49.2 (27.1-71.8) ng/mL] compared to healthy controls [58.5 (27.3-80.8) ng/mL] (P < 0.001). Infants with zonulin levels ≤55.15 ng/mL had 11.64 times more risk of developing AD than the infants with zonulin levels >55.15 ng/mL. Infants whose MEC/CCL28 levels were ≥8.3 ng/mL had 5.83 times more risk of developing AD than the infants with MEC levels <8.3 ng/mL. Duration of AD and SCORAD index score did not show correlation with MEC/CCL28, TECK/CCL25, periostin and zonulin levels. Conclusion: Low zonulin levels and high MEC/CCL28 levels in infants may show an increased association with AD.

Cephalometric Mandibular Dimensions in Growing Turkish Children: Trends of Change, Sex-Specific Differences, and Comparisons with Published Norms.

OBJECTIVE: The aims of this study were to investigate cephalometric mandibular dimensions in growing Anatolian Turkish children and to identify the periods of rapid growth for boys and girls. Furthermore, the secondary aim was to compare obtained values with published standards in the literature. METHODS: A total of 528 pretreatment lateral cephalometric radiographs, grouped according to age and sex, were analyzed. Effective mandibular length, ramus height, and corpus lengths were comparatively evaluated within age groups for boys and girls and between sexes for the same age group. Data acquired from this study were compared with American, Canadian, Chinese, and European norms. Growth curves for mandible were constructed for each sex group. RESULTS: Effective mandibular length was almost always significantly longer in boys, except for 9- and 12-year-age groups. Effective mandibular length in girls increased significantly between ages 8 and 10, 10 and 12, and 11 and 13 years, while in boys between ages 8 and 10, 9 and 11, and 13 and 15 years. Turkish girls had significantly shorter effective mandibular lengths than American girls at age 14. No significant difference was found between Turkish and Chinese girls and boys. Turkish girls and boys had significantly shorter corpus lengths from their Norwegian counterparts at age 12. CONCLUSION: Except for 9- and 12-year-age groups, effective mandibular length was almost always significantly longer in boys compared to the girls. It is suggested to use norm values from more recently conducted studies and which are representative of the studied population. Growth curves can be used to predict the approximate mandibular dimensions at a particular age.

Intensive physiotherapy versus home-based exercise and custom-made orthotic insoles in patients with plantar fasciitis: Pilot study.

BACKGROUND: This prospective pilot study assessed the feasibility of comparing intensive physiotherapy group (IPG), home-based exercise (HBEG) group, and insole group (IG) to find the most effective treatment program for plantar fasciitis. METHODS: Thirty-nine individuals with plantar fasciitis were selected to participate in this pilot study. The patients were randomly divided into 3 groups, as IPG (manual interventions, exercise, insoles), HBEG (exercise, insoles), and IG (insoles). The feasibility details including percentage of the enrollment and adherence to the groups were recorded. Pain was measured with a visual analog scale, and dorsiflexion range of motion (DROM), foot function, and health-related quality of life were evaluated at the baseline and week 6. One-way ANOVA analysis and Kruskal Wallis test were used to determine the differences of the percentage change of the parameters. RESULTS: Pain and functional evaluation results showed improvements clinically in all of groups and quality of life results were similar for all groups. There was no difference between the groups after 6 weeks of treatment (P > 0.05). CONCLUSIONS: The 3 treatment programs were found clinically applicable with high patient adherence. According to the data all intervention types were found moderately effective for pain and function. Insoles could be an option applied alone or with other treatments, and manual techniques gave better results in patients with limited ankle dorsiflexion. Although the results should be interpreted with caution because of the small sample size, these results were found promising and feasible to conduct a future RCT. CLINICAL TRIAL REGISTRATION NUMBER: RCT 06144834.

Absence of TFE3 Immunoexpression in a Spectrum of Cutaneous Mixed Tumors: A Retrospective Pilot Study.

BACKGROUND: Cutaneous mixed tumors (CMTs) include benign, atypical, and malignant chondroid syringomas. This spectrum of entities is known to be a part of myoepithelial neoplasms, which display considerable genetic heterogeneity. In a previous report, a malignant chondroid syringoma (MCS) demonstrated PHF1-TFE3 gene fusion and strong TFE3 immunohistochemical (IHC) staining. The authors suggested that the MCS is genetically related to tumors with TFE3 rearrangements such as renal cell carcinoma and might have genetic heterogeneity. In this study, we aim to investigate potential TFE3 gene fusions with TFE3 IHC stain in a spectrum of CMTs. MATERIALS: Eleven benign chondroid syringoma (BCS), one atypical chondroid syringoma (ACS), and one malignant chondroid syringoma cases were identified, stained with TFE3 IHC stain, and interpreted based on preset criteria. RESULTS: ACS and MCS cases did not show any staining. In 7 of 11 BCS cases, weak (1+) staining was observed in less than 20% of the tumor cells and were considered negative. Additionally, in one BCS case, weak (1+) and (2+) staining was shown in approximately 15% and less than 1% of the tumor cells, respectively. Based on our positivity criteria, this case was also interpreted as negative. CONCLUSIONS: Our study failed to reveal possible TFE3 gene fusion by IHC staining in benign, atypical, and malignant chondroid syringomas. Although the negative staining in MCS suggests a genetic heterogeneity in this entity, further studies with larger case groups are needed for a more definitive conclusion.

Assessment of dental anxiety levels among dental emergency patients during the COVID­19 pandemic through the Modified Dental Anxiety Scale.

BACKGROUND: Coronavirus disease 2019 (COVID­19) continues to affect dental emergency services worldwide. Dental anxiety (DA) is described as a common and distressing problem in terms of oral health maintenance. OBJECTIVES: The present study aimed to evaluate DA levels as well as the COVID­19 fear and perception of control (COVID­19 FPC) in patients attending dental emergency clinics during the COVID­19 pandemic. MATERIAL AND METHODS: Sociodemographic, dental and medical data was obtained from the participants. A face-to-face questionnaire with questions referring to the reasons for the emergency dental visit, the visual pain scale, the Modified Dental Anxiety Scale (MDAS), and the COVID­19 Fear and Perception of Control Scale (COVID­19 FPCS) as well as additional questions concerning bruxism and a previous diagnosis of anxiety/panic attacks or depression was administered. The χ2 test was used to analyze the data. RESULTS: A total of 1,439 patients were included in the study. The most common reason for the dental visit was pain (47.5%). The prevalence of DA was 5.1% (74/1,439). A significant association was found between DA and gender (p = 0.020). The incidence of severe pain was higher in patients with DA than in those without DA (p = 0.002). No significant differences in the MDAS scores were found between patients with and without a chronic disease (p = 0.804), with regard to the educational status (p = 0.364), or between the age groups (p = 0.600). The prevalence of a 'strongly agree' response to all questions in COVID­19 FPCS was higher in patients with DA as compared to those without DA. CONCLUSIONS: Females and patients with severe pain were more likely to exhibit DA. In general, patients with DA strongly agreed with the statements of COVID­19 FPCS, which may indicate a correlation between the 2 scales.

A Young Boy With Hemophagocytic Lymphohistiocytosis Presenting With Vaccine-Related Granulomatous Dermatitis: A Case Report and Literature Review.

ABSTRACT: Cutaneous eruptions associated with hemophagocytic lymphohistiocytosis (HLH) have been reported in 6%-63% of patients. Clinical findings of these skin lesions vary widely and include maculopapular rashes, ulcers, and violaceous nodules. Corresponding histologic findings are also variable and are considered nonspecific. We report the case of a 4-year-old boy who initially developed a widespread popular-pustular rash 2 weeks after his 12-month measles, mumps, and rubella vaccinations. These resolved with scarring then recurred following his 24-month vaccinations. Multiple skin biopsies were negative for infectious organisms and showed a granulomatous infiltrate with perforation and necrobiosis. The differential diagnosis included perforating granuloma annulare, infection, or rheumatoid nodules. At the age of 4, he developed fever, hepatosplenomegaly, pancytopenia and other laboratory abnormalities, requiring hospitalization. A number of studies were performed including biopsies of bone marrow and liver. Molecular testing revealed 2 mutations in UNC13D known to be associated with familial HLH. His prior cutaneous lesions were likely caused by immune dysregulation exacerbated by immunizations because of underlying familial HLH. This case illustrates the importance of recognizing an unusual cutaneous manifestation of a rare disease to arrive at an earlier diagnosis in a pediatric patient. Although cutaneous eruptions usually develop concurrently with other systemic symptoms of HLH, preceding unusual skin lesions may be the first indication of this rare disease.