DIAGNOSTIC CRITERIA AND CLINICAL COURSE OF ATOPIC DERMATITIS IN ADULTS

Objective: to analyze Atopic dermatitis (AD) diagnostics criteria routinely used for diagnosis as well as common findings and history in adult patients with different age of onset of the disease. Materials and methods. 123 adult patients aged between 18 and 58 years with AD relapse were examined. The diagnosis was confirmed according to Ukrainian guidelines on AD. The patients were randomized in 2 groups depending on AD age of onset: 67 patients had AD start in early childhood and puberty, 56 patients – in adulthood. Precise medical history of the patients, clinical symptoms were collected. The disease severity was evaluated with SCORAD index and itch severity scale. Results were analyzed using parametric and nonparametric statistical tools. Results. Depending on age of AD onset patients demonstrated some differences in diagnostic criteria positivity, past and actual clinical features of the disease, cooperation with health system specialists. Among major AD diagnostic criteria personal or family history of atopy and flexural lichenification in adults were detected as variable signs of the disease depending on age of onset. Family history of atopy stayed hidden information for part of the patients and could not be verified. The most often minor criteria detected positive and used for AD diagnosis in adults were xerosis, itching when sweating, facial pallor or erythema and white dermographism, with their different frequency among groups. 17,9% of cases in childhood and 25% of cases in adults were not initially recognized as AD and managed as another skin disease for months or years. Skin itch as constant sign of AD is provoked by different triggered with high prevalence of emotional stress in adult-onset AD and different frequency of contact triggers among groups. Emotional trauma was considered by the patients as a major trigger of mild relapses in adult-age AD and moderate relapses in early-age AD. Constant and severe xerosis was more common for patients with AD onset in childhood and was mostly not a significant impacting issue for adult-aged AD. Secondary pyoderma accompanies AD relapses of both groups. 100% of examined patients had experience of self-treatment with topical and systemic medications. Physical modalities of treatment (sunbathing, change of climate etc.) are less often of adult-age AD patients’ choice and provide good results more for early onset AD patients. Steroid-phobia was observed in patients with early disease onset only and is not an issue for adult-age AD patients. Conclusions. Diagnostic criteria stay an important tool for diagnosing AD. Adult patients may demonstrate different set of positive major and minor criteria depending on time of the disease onset. Established diagnosis of AD may not correspond to first manifestation of the disease that is possible both in childhood and adult patients. Prognosing of AD course in adults may depend on general duration of the disease: starting in childhood AD more often has seasonal relation, constant xerosis, irritation by sweeting; patients have long experience of moisturizers and physical treatments use. If started in adulty AD more often aggravates due to emotional and occupational triggers, then others; long-term use of moisturizers more probably would be neglected by the patient.

600 Validation of the academic research consortium high bleeding risk criteria in patients undergoing percutaneous coronary intervention: a systematic review and meta-analysis of 10 studies and 67 862 patients

Aims The Academic Research Consortium for High Bleeding Risk (ARC-HBR) has recently proposed, by consensus, 20 clinical criteria for the assessment of the bleeding risk after percutaneous coronary intervention (PCI). A major criterion was defined as any individual clinical condition conferring in isolation a risk for major bleeding ≥4% up to 1 year after PCI; instead, a minor criterion was considered to confer a bleeding risk of < 4%. The aim of this meta-analysis was to assess the performance of the ARC-HBR criteria in stratifying the risk of bleeding and ischaemic events after PCI. Methods and results MEDLINE, COCHRANE, Web of Sciences, and SCOPUS were searched for studies aimed at validating the ARC-HBR criteria in patients treated with PCI. The primary outcome measure of this meta-analysis was major bleeding. The analysis included 10 studies encompassing 67 862 patients undergoing PCI; the HBR definition was fulfilled in 44.7% of the cases. The risk of major bleeding was significantly higher in HBR vs. Non-HBR group (RR: 2.56, 95% CI: 2.28–2.89). The average C-statistic was 0.64 (95% CI: 0.60–0.68), indicating modest discrimination. The risk of intracranial haemorrhage, gastrointestinal bleeding, fatal bleeding, ischaemic stroke, cardiac death and all-cause death was higher in HBR vs. Non-HBR group. Despite a higher incidence of myocardial infarction and stent thrombosis in patients deemed at HBR, the rate of target lesion revascularization was comparable between groups (RR, 1.01, 95% CI: 0.88–1.16). When assessed in isolation, the mean cumulative incidence of major bleeding at 1 year exceeded the cut-off value of 4% for all the major criteria and for two out of six minor criteria, including age ≥75 years and moderate chronic kidney disease (CKD) (Figure). Conclusions The ARC-HBR definition identifies patients at higher risk of major bleeding and other adverse cardiovascular events after PCI. Almost all major criteria, but also two of the minor criteria, were individually associated with rates of major bleeding above 4% thus fulfilling the definition of major HBR criteria. These findings corroborate the ability of ARC-HBR major criteria in identifying PCI patients who are more likely to develop adverse events, but also suggest caution in the decision making of patients with isolated minor criteria, including age ≥ 75 years and moderate CKD.

Classical and Non-Classical Phenotypes of Erdheim-Chester Disease: Correlating Clinical, Radiographic, and Genotypic Findings

Background: Erdheim-Chester disease (ECD) is characterized by multi-organ infiltration of clonal histiocytes bearing activating mutations predominantly in the MAPK pathway. The diagnosis of ECD is clinico-pathologic; histopathologic findings alone are often non-specific. Characteristic pathognomonic finding of ECD is the symmetric osteosclerosis of the distal femur and proximal tibia/fibula, seen in >90% of cases, and referred to herein as classic ECD (C-ECD). There is a paucity of data on the phenotypic and mutational differences between C-ECD and non-classic ECD (NC-ECD). Determining phenotypic patterns may allow for earlier suspicion and diagnosis. Methods: Patients who met the revised ECD criteria proposed by Haroche J et al (Blood 2020;135:1311-1318) and had full body imaging that included the lower legs (18-FDG-PET/CT or CT/bone scan) were included. ECD diagnosis was made when >1 major criteria plus >1 minor criteria were present. Major criteria: 1) symmetric meta-diaphyseal osteosclerosis in legs; 2) "hairy kidneys"; "coated aorta", right atrial pseudotumor, xanthelasma, exophthalmos; or osteosclerosis of paranasal sinuses. Minor criteria: 1) histologic finding of typical foamy histiocytes (CD68+/CD163+/CD1a-) associated with fibrosis; 2) mutation/gene fusion of BRAF, CSF1R, or MAPK/PI3K pathways. We compared the organ involvement and BRAF V600Emutational status between C-ECD and NC-ECD. Results: A total of 105 patients were included. The median age at diagnosis was 57 years (range, 38-81) and most were males (62%). Majority had 18-FDG-PET/CT (83%) and BRAF V600E testing (65%). The main organ systems involved were skeletal (83%), renal (64%), adrenal (44%), and pulmonary (42%). Central diabetes insipidus (DI), "hairy kidneys", and "coated aorta" were present in 27 (26%), 54 (51%), and 44 (42%) patients, respectively. Among those tested, BRAF V600E mutation was found in 48/67 (72%) by immunohistochemistry. In our cohort, most patients (n=87. 83%) had C-ECD. NC-ECD had significantly lower number of organs/systems involved compared with C-ECD (median 3 vs 6, p=0.002). C-ECD had significantly higher rates of involvement of paranasal sinuses (51%/7%, p=0.002), DI (26%/0%, p=0.02), and similar rates of lung (44%/43%), cardiac (34%/14%), and skin (14%14%) involvement when compared to NC-ECD. BRAF V600E was significantly more common in C-ECD (88%/30%, p=0.004). Thirty-nine (37%) patients underwent next generation sequencing, of whom 33 (31%) had successful testing. In C-ECD, 3 patients had mutations other than BRAF V600E, these included: NRAS, MAP2K1, and MEF2C-FLT3 fusion. In NC-ECD, 5 patients had mutations other than BRAF V600E, these included: MAP2K1, KRAS, and NF1. Conclusions: Our study suggests distinct differences in clinical presentation and molecular findings exist between C-ECD and NC-ECD. C-ECD has a higher degree of organ involvement and harbor BRAF V600E more frequently than NC-ECD. Further analysis of histopathologic findings and outcomes in this cohort may provide insights into these ECD subsets that can optimize future management of this disease. Figure 1: Sites of involvement of classical Erdheim-Chester Disease (C-ECD) versus non-classical Erdheim-Chester Disease (NC-ECD) Figure 1 Figure 1. Disclosures Vassallo: Bristol-Myers-Squibb: Research Funding; Sun Pharma.: Research Funding; Pfizer: Research Funding. Tobin: Mayo Clinic Center for MS and Autoimmune Neurology: Research Funding; Mallinckrodt Pharmaceuticals: Research Funding; National Institutes of Health: Research Funding. Bennani: Verastem: Other: Advisory Board; Purdue Pharma: Other: Advisory Board; Vividion: Consultancy, Other: Advisory Board; Daichii Sankyo Inc: Other: Advisory Board; Kyowa Kirin: Other: Advisory Board.

Abstract 1122‐000111: Embolic Stroke of Undermined Significance with Unusual Degree of CSF Neutrophilic Pleocytosis

Introduction : A 50 year old African American female with a history of hyperlipidemia, hypertension, diabetes mellitus, and peripheral artery disease with right lower extremity bypass earlier in 2021 presented with altered mental status (AMS) and right‐sided facial droop. She presented to an outside hospital where her temperature was 102.1°F and blood pressure was 185/84. The National Institute of Health Stroke Scale (NIHSS) was 16. Notable labs included white blood cell (WBC) count 10.3, sodium 133, lactate 2.7. Urine drug screen notable for THC. Urinalysis, CXR, COVID screen were negative. CT of the head did not show acute findings, CT angiogram did not show any stenosis or large vessel occlusions and CT perfusion revealed perfusion deficits in the left hemisphere. Given her elevated temperature and lactate, a lumbar puncture (LP) was performed. Cerebrospinal fluid (CSF) analysis revealed WBC count 58 (95% neutrophilic predominance), RBC count 128, glucose 324 (serum glucose 576), protein 77 and lactate dehydrogenase (LDH) 23. Concerns for meningitis lead to her being started on broad spectrum antibiotics (ampicillin, ceftriaxone, acyclovir, and vancomycin). She was then transferred to our comprehensive stroke center for further management. Methods : Initially she remained febrile and somnolent, but after 36 hours of antibiotics, her mentation improved. Antibiotics and antivirals were slowly tapered after the CSF meningitis panel, gram stain, cultures, and viral PCRs came back negative. MRI of brain showed acute left posterior cerebral artery (PCA) ischemic stroke with punctate infarcts of right lentiform nucleus and periventricular area. Transthoracic echocardiogram (TTE) showed the left ventricle with severe hypertrophy and ejection fraction (EF) 65–70%. There was concern for endocarditis with systemic infection, however transesophageal echocardiogram (TEE) was negative for infectious vegetations and bubble study was negative. Blood cultures showed no growth after four days. Syphilis screen, ANA, HIV were also negative. Lipids were elevated with total cholesterol 214 and LDL 138. Hemoglobin A1c was also elevated at 13. Results : After being stable for several days, the patient’s NIHSS reduced to three, two points for right homonymous hemianopia and one point for minor facial paralysis. An implantable loop recorder was placed to monitor for any arrhythmias that may have led to her stroke and the patient was discharged home on aspirin and atorvastatin. Conclusions : With the initial presentation of fever and AMS in this patient, there was high suspicion of infective endocarditis. She also suffered an ischemic stroke which was determined to be embolic from an undetermined source. The patient did not meet modified Duke Criteria for “possible infective endocarditis” which is considered when the patient has one major and one minor criteria or three minor criteria. Two minor criteria were met including a temperature > 38oC on admission and vascular embolic phenomena (stroke). Interestingly, blood and CSF cultures never grew an organism although the CSF WBC count was 58. While CSF lymphocytosis has been associated with TIA‐like presentations and other viral or fungal etiologies associated with ischemic stroke, this is perhaps the first case of a neutrophilic‐predominant CSF pleocytosis in setting of ischemic stroke without a clear source.

The Myelic Limited Dorsal Malformation: Prenatal Ultrasonographic Characteristics of an Intermediate Form of Dysraphism

<b><i>Objectives:</i></b> The aim of the study was to report a subtype of dysraphism designated as myelic limited dorsal malformation (MyeLDM) and to describe its characteristics at prenatal ultrasound (US). <b><i>Methods:</i></b> It was a retrospective study from 2014 to 2020 based on second-line US evaluation of patients referred to our institution for myelomeningocele (MMC). Magnetic resonance imaging and acetylcholine esterase evaluation in the amniotic fluid were also offered. Major and minor criteria for open and closed dysraphism were defined and recorded for each patient. Patients were included as MyeLDM when both criteria of closed and open dysraphism were observed in the same fetus. Correlations were obtained with the postpartum data. <b><i>Results:</i></b> Twenty patients fulfilled the inclusion criteria, some of them being very close to MMC, others very close to limited dorsal myeloschisis (LDM), and others lying in between. There were 13 live-born neonates and 7 terminations of pregnancy. Correlations between prenatal and postpartum data were overall very good. <b><i>Conclusion:</i></b> Our series describe the ultrasonographic characteristics of an intermediate type of dysraphism and suggest that there is a continuum between MMC and LDM with numerous possibilities of hybrid forms (MyeLDM) sharing characteristics of both open and closed dysraphisms.

Frequency of high bleeding risk in patients undergoing percutaneous coronary intervention

Objective: To assess the prevalence of risk factors for bleeding and high bleeding risk in patients undergoing percutaneous coronary intervention. Method: The single-centre retrospective observational study was conducted at ?? and comprised data of patients who underwent percutaneous coronary intervention from February 2018 to December 2019. Minor and major bleeding risk factors were identified on the basis of the consensus definition of the Academic Research Consortium. Patients with high bleeding risk were identified according to the consensus-based criteria of at least one major criterion or two minor criteria. Data was analysed using SPSS 20. Results: Of the 385 patients, 280(72.7%) were males. The overall mean age was 57.9+/-11.9 years. The indication of procedure was acute coronary syndrome in 367(95%) patients. Of all the patients, 171(45%) had bleeding risk, with 94(24%) patients having a high bleeding risk. Of these, 60(15.6%) patients had high risk based on the presence of at least one major criterion and 34(8.8%) patients because of the presence of two or more minor criteria. Patients with high bleeding risk were more likely to be older and female with more co-morbidities (p<0.05). Conclusion: Almost half of the patients undergoing percutaneous coronary intervention were found to have at least one bleeding risk factor, and one in four patients had high bleeding risk. Key Words: Haemorrhage, Dual antiplatelet therapy, Percutaneous coronary intervention. Continuous...

Histological Patterns of Skin Lesions in Tuberous Sclerosis Complex: A Panorama

Tuberous Sclerosis Complex (TSC) is a multisystem genetic disease characterized by cutaneous and extracutaneous hamartomas. The diagnosis is based on the association of major and minor criteria, defined by a consensus conference updated in 2012. The clinical examination of the skin is crucial because seven diagnostic criteria are dermatological: four major (hypomelanotic macules, angiofibroma or fibrous cephalic plaques, ungual fibromas, shagreen patches) and three minor criteria (confetti skin lesions, dental enamel pits, intraoral fibromas). Skin biopsy is commonly performed to assert the diagnosis of TSC when the clinical aspect is atypical. Histopathology of TSC cutaneous lesions have been poorly reported until now. In this article, we review the histologic features described in the literature and share our experience of TSC skin biopsies in our pediatric hospital specialized in genetic disorders. Both hypomelanotic lesions and cutaneous hamartomas (angiofibroma/fibrous cephalic plaques, ungual fibromas, shagreen patches) are discussed, including the recent entity called folliculocystic and collagen hamartoma, with a special emphasis on helpful clues for TSC in such lesions.

Unusual presentation of infective endocarditis in ICU

Bacterial endocarditis remains a challenging condition to manage owing to its variety of different presentations. This report describes a 55-year-old woman with endocarditis who presented confused with shoulder and back pain. Initial diagnosis was made difficult by a negative echocardiogram but aided by striking peripheral stigmata. She was treated for infective endocarditis as she met all five Duke’s minor criteria for infective endocarditis. Gallium scan was a useful investigation in identifying lumbar spine and acromioclavicular joint septic foci. This case highlights the challenges of diagnosing endocarditis. It also describes how gallium scans can be useful in identifying occult septic emboli in these patients.