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Objectives: The 90-day double-blind phase (DBP) of the Phase 3 EASE study demonstrated accelerated wound healing for Oleogel-S10 (birch triterpenes) versus control gel in epidermolysis bullosa (EB). Here, we report safety and total wound burden results from the 24-month open-label phase (OLP) in which all patients received treatment with Oleogel-S10. Method(s): Total wound burden was assessed using EB Disease Activity and Scarring Index (EBDASI) and Body Surface Area Percentage (BSAP). Data are reported without visit windows to reflect a realworld situation more accurately, particularly considering the COVID- 19 pandemic. Result(s): The patient population was made up of dystrophic EB (n = 178, 86.8%) and junctional EB (n = 25, 12.2%);71.7% (n = 147) of patients were aged <18 years. 141 patients (68.8%) completed the OLP. The mean (SD) treatment duration for all patients was 584.7 (246.1) days. Adverse events were reported in 77.1% of all patients in the OLP versus 81.7% of those receiving Oleogel-S10 in the DBP. Mean BSAP for patients treated with Oleogel-S10 in the DBP reduced from 12.1% at study entry to 6.1% with 27 months of treatment. Similarly, the mean EBDASI skin activity score in the Oleogel-S10 group improved from 19.6 to 15.1 after 27 months. In addition, reductions in both BSAP and EBDASI from OLP baseline were observed in patients who transitioned from control gel to Oleogel-S10 in the OLP. Discussion(s): These data support a reassuring long-term safety profile of Oleogel-S10. Furthermore, the reduction in wound burden previously reported with 15 months of Oleogel-S10 treatment is maintained to the end of OLP. This is encouraging given the nature of this chronic genetic disorder in which there is regular cycling of patients' fragile wounds.
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Background/Aims Advances in rational drug design and recent clinical trials are leading to emergence of a range of novel therapies for SLE and therapeutic options in clinical practice are expected to broaden rapidly. The optimal real-world place of emerging and established agents will be guided by understanding their differential efficacy on specific SLE manifestations as well as efficacy for more resistant disease. Anifrolumab, a type-I interferon receptor blocking monoclonal antibody, showed efficacy in SLE in phase III trials with a notable effect on mucocutaneous disease although specific lesion subtypes and chroncicity were not explored. Severe refractory mucocutaneous SLE such as scarring discoid lesions are an important and common clinical challenge in current practice. We therefore prospectively evaluated the real-world efficacy and quality of life impact of anifolumab for active mucocutaneous SLE, recalcitrant to multiple biologic and immunosuppressant therapies. Methods Seven patients commenced anifrolumab (300mg by monthly iv infusion) following application to the manufacturer's early access programme (NCT 04750057). Prior biologic therapies were discontinued at least 5 half-lives in advance. Mucocutaneous disease activity was captured by Cutaneous Lupus Disease Area and Severity Index (CLASI) activity score and medical photography. Patient reported health-related quality of life comprising the Dermatology Life Quality Index (DLQI);Lupus-QoL and EQ5D-5L were evaluated at baseline, three and six months. Results Seven female patients with active mucocutaneous SLE (Discoid LE n=5, chilblain LE n=1, subacute cutaneous LE n=1) and median disease duration of 17 years were evaluated. Median baseline CLASI activity score was 17 (range 10-26;higher scores indicating severe disease). Median number of previously failed therapies was 7 and included rituximab in 6/7, belimumab in 2/7 and thalidomide in 4/7. Rapid resolution of scale and erythema in DLE was established within 1 month of anifrolumab treatment. Improvements to chilblain lupus were evident by three months. CLASI activity score was improved >=75% in all patients at 3 months. Clinical responses were associated with significant improvements in DLQI (p<0.001) and EQ5D-VAS (p=0.002) by three months. Lupus-QoL trended toward improvement across all domains but most strongly for fatigue (p=0.01) and pain (p=0.002) by 6 months. One patient discontinued treatment after 4 months due to polydermatomal shingles complicated by sensorineural hearing loss. Infection coincided with background prednisolone dose >15mg daily, recent COVID-19 infection and new on-treatment hypogammaglobulinaemia (IgG <5g/L). Prolonged aciclovir treatment was required for lesion resolution. Conclusion We report rapid real-world efficacy and quality of life impact of anifrolumab on highly refractory mucocutaneous SLE, which exceeded that anticipated from existing clinical trial data. Findings suggest a unique role for emerging interferon targeting therapies in management of mucocutaneous SLE but emphasize need for enhanced VZV precautions among higher risk patients.
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In 2022, mpox virus spread globally with 99% of cases in non-endemic countries. People living with HIV (PLHIV) are disproportionally affected, often with more severe clinical features and outcomes. The AAD/ILDS Dermatology COVID-19, Monkeypox (mpox), and Emerging Infections registry captured mpox cases from 13 non-endemic countries in a de-identified REDCap registry. We aimed to examine cutaneous symptomatology and outcomes in cases of mpox in PLHIV. Of 119 reported cases, 44 were PLHIV (35%). Cases were 98% male, with a median age of 38 years, located in Europe (57%) and the U.S. (39%). Nearly half of PLHIV reported skin lesions as their initial sign (45%), and 43 (98%) reported skin lesions during illness. The primary initial lesion locations were peri-anal (34%) and genito-inguinal (34%). Co-infection with other sexually transmissible infections (STI) was more common in PLHIV, 57% vs. 38% in all-registry cases (p<0.01). The most common co-infections were gonorrhea, syphilis, and chlamydia. Time to resolution was 17 days, 3 days shorter than all-registry cases, which may be due to higher use of Tecovirimat in PLHIV (36% vs. 25% in all-registry cases;p<0.01). There were no differences in the frequency of hospitalization or scarring. One death was reported. Overall, cutaneous lesion count was similar in PLHIV and all-registry cases. Lesion location was more frequently reported in the peri-anal and genito-inguinal regions. Sample size was insufficient to detect differences in length of infection, hospitalization, or scarring in PLHIV. Co-infections were more common in PLHIV, highlighting a need for co-testing for STIs during mpox evaluation.Copyright © 2023
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A 58-year-old African woman was referred for the management of scalp alopecia that had started in her thirties over the vertex of her scalp and had progressively involved > 90% of her scalp over the years. She was diagnosed with central centrifugal cicatricial alopecia of many years' duration and was being managed with annual wigs. On further questioning in the clinic, her chief complaint was a painful area over the vertex of the scalp, which had been affecting her sleep for years. She admitted to having used hair treatments in the past, after her hair loss had started. Clinical examination revealed a soft, tender, boggy swelling at the site of pain;however, the rest of her scalp felt like palpating cotton wool. Trichoscopic examination showed no remnant follicular openings, and histology showed end-stage alopecia. Her body mass index (BMI) was noted to be > 45 and there was an associated ichthyosiform rash on her shins bilaterally. Magnetic resonance imaging of her brain was requested for suspected lipoedematous scalp alopecia. Radiological findings led to a diagnosis by demonstrating 24-mm-thick subcutaneous adipose tissue - the highest recorded for this condition. Normal scalp thickness is suggested to be 6 mm. The patient declined a trial of intralesional steroids and topical Dermovate was unsuccessful. Lipoedematous scalp is a rare disease marked by a soft thickening of the scalp, first recorded in 1935 (Cornbleet T. Cutis verticis gyrata? Lipoma? Arch Dermatol Syphilol 1935;32: 688). A similar clinicopathological entity associated with nonscarring but permanent acquired alopecia was described in 1961 (Coskey RJ, Fosnaugh RP, Fine G. Lipedematous alopecia. Arch Dermatol 1961;84: 619-22) and termed lipoedematous alopecia. Our case was associated with a raised BMI and also with an ichthyosiform rash on her shins, which started when she was in her teens and needs further evaluation after the COVID-19 pandemic. It was also associated with scarring alopecia rather than nonscarring alopecia, as seen in other cases. We wonder if there is a genetic cause as the disease predominantly occurs in African patients. Also, lipoedematous alopecia may be associated with nonscarring alopecia, as well as scarring alopecia, as seen in our case.
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Aplasia cutis congenita (ACC) is often sporadic, but familial cases have been reported. We report a case of a dichorionic diamniotic twin pregnancy in which both the male and female twins had matching areas of aplasia cutis on their scalps. An Irish couple sought fertility treatment using a donor egg and paternal sperm. Successful in vitro fertilization (IVF) and the transfer of two embryos resulted in a diamniotic dichorionic twin pregnancy. Two fetal poles were noted at the 12-week ultrasound (US) scans. The mother suffered from a minor urinary tract infection during the first trimester but had no other history of infection, including herpes simplex virus or COVID- 19. She was known to be varicella immune prior to pregnancy. The twins were born by elective caesarean section owing to breech presentation. Twin one was female and twin two was male. Both infants were born with scarring on the crown of their head, which was consistent with ACC. Cranial US showed no underlying bony abnormality. The rest of the cutaneous examination was normal and there were no other congenital anomalies. ACC is a rare, heterogeneous group of disorders characterized by the congenital absence of skin, which can be focal or widespread. It is thought to affect 1-3 per 10 000 live births. The exact cause of ACC is unclear. Various hypotheses have been suggested, including defective closure of the neural tube or embryonic fusion lines, intrauterine trauma, placental insufficiency, fetus papyraceus, amniotic membrane adhesions, intrauterine infections, teratogens and genetic mutations. The classification of ACC is based on the area affected, type of skin irregularity, associated congenital defects and mode of inheritance. Scalp ACC without multiple anomalies (category 1) is generally associated with an autosomal dominant or sporadic pattern of inheritance. These twins may have an autosomal dominant mutation that led to this phenotype. ACC can also be associated with fetus papyraceus or placental infarct. This is less likely in this case as only two embryos were transferred, and the pregnancy was dichorionic. Most cases of ACC associated with fetus papyraceus occur in monozygotic pregnancies. ACC lesions often heal spontaneously by re-epithelialization resulting in a hairless superficial scar. Twin one had a slightly smaller area affected by ACC and overlying eschar resolved several weeks after birth. Twin two has had no hair growth in the area. This case highlights the difficulties in ascertaining the aetiology of this rare condition in twin pregnancies.
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Background Dilated cardiomyopathy (DCM) is caused by many conditions, including ischemia, genetics, infection, chemotherapy, or idiopathic. Clinical suspicion is needed to identify reversible etiologies. Case A middle-aged truck-driver presents with exertional dyspnea, cough, lower extremity edema, and low-grade fever for 2 weeks. He had 20-pack-year smoking history and 3-4 alcoholic drinks weekly. Chest x-ray showed pulmonary congestion. BNP was elevated. ECHO showed severely dilated ventricles with LVEF < 10% and no wall abnormalities. Decision-making Perfusion stress test showed no inducible ischemia. Coronary angiogram showed no epicardial disease. Cardiac MRI (CMR) showed severely dilated biventricular failure, pericardial thickening, circumferential pericardial effusion, epicardial involvement suggestive of subacute myopericardial inflammation and scarring with delayed gadolinium-enhancement and RVEF < 5%. Liver ultrasound showed no cirrhosis. Viral PCR was positive for rhinovirus, negative COVID-19. He was treated medically requiring inotropes then transferred to heart failure center for assist device evaluation. Conclusion Our patient reported moderate alcohol use, which alone would not explain the myopericardial changes seen on CMR. Given the findings, his DCM was attributed to alcohol complicated by possible subacute rhinovirus myocarditis. Our association is further supported by recent respiratory viral prodrome along with exclusion of other etiologies. [Formula presented]Copyright © 2023 American College of Cardiology Foundation
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The inception of the COVID-19 pandemic has jeopardized humanity with markedly dam-pening of worldwide resources. The viral infection may present with varying signs and symptoms, imitating pneumonia and seasonal flu. With a gradual course, this may progress and result in the deadliest state of acute respiratory distress syndrome (ARDS) and acute lung injury (ALI). More-over, following recovery from the severe brunt of COVID-19 infection, interstitial portions of alve-oli have been found to undergo residual scarring and further to have compromised air exchange. Such alterations in the lung microenvironment and associated systemic manifestations have been recognized to occur due to the extensive release of cytokines. The mortality rate increases with advancing age and in individuals with underlying co-morbidity. Presently, there is no availability of specific antiviral therapy or any other definitive modality to counter this progressive worsening. However, we believe principles and advancing cell-based therapy may prove fruitful in subjugating such reported worsening in these patients. This article reviews eminent knowledge and relevant ad-vancements about the amelioration of lung damage due to COVID-19 infection using adipose tis-sue-derived-total stromal fraction (TSF).Copyright © 2022 Bentham Science Publishers.
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SARS-CoV-2 has affected essentially all countries worldwide and caused millions of people to become infected and die. Therefore, it is extremely valuable to investigate new approaches to stop the most scarring ongoing pandemic. BCG vaccine has been proposed that it could reduce the rate of new COVID cases and limit the severity of infection since TB and COVID-19 have similar dominant effects, such as cytokine storm and improper immune response. This review aimed to focus on the latest literature data on trained immunity as well as the possible cross protection effect of BCG vaccine against COVID-19. The first immune response to BCG vaccines has started with the stimulation of adaptive immune response and establishment of the immunological memory of antigen-specific T and B cells to target infectious agents. In the past years, innate immune response was thought to be not having the talent to adapt and "learn” from previous exposure to a pathogen. Trained immunity is conceivable as 'de facto' innate immune system memory. Some researches argue that there is a strong relationship between BCG immunization and COVID-19 although some are against this argument. Based on the data obtained from different research studies and ongoing clinical trials, there is still no evidence that BCG vaccine is effective against COVID-19. Besides assumptions, knowns and unknowns, the clinical efficiency of BCG vaccine against SARS-CoV-2 should be validated by accurate scientific clinical reports in different age groups to understand the potential benefits of BCG vaccine to limit COVID-19 incidence and mortality.
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Case Report: Tsukamurella species are aerobic, partially acid fast saprophytes commonly isolated from soil and water. They are opportunistic pathogens known to infect multiple organs and can contribute to significant pathologies such as bacteremia, peritonitis, and respiratory tract infections. Moreover, Tsukamurella shares certain characteristic properties to Mycobacterium tuberculosis and Actinomyces species, including the acid fast stain, which can contribute to misdiagnosis of patients. A 68 year old female patient presented to the ED for shortness of breath, fatigue, and weight loss for 6 months. The patient's past medical history includes pulmonary fibrosis, type 2 diabetes, coronary artery disease with stent, hyperlipidemia, hypertension, and M. tuberculosis infection when she was 3 years old in Finland. On admission, labs revealed thrombocytosis (reactive 555 000/microL), leukocytosis (14 450/microL), and microcytic anemia (9.4 microg/dl). Moreover, C reactive protein was elevated and procalcitonin was normal (0.06 microg/l);a COVID-19 PCR was negative. An X-ray revealed severe patchy and interstitial infiltrates throughout both lungs with parenchymal scarring and pleural thickening in the periphery of the left mid-lung zone with multifocal pneumonia. Blood and sputum cultures were performed under the impression of pneumonia, and treatment with azithromycin and ceftriaxone was started. A M. tuberculosis infection was suspected due to a positive AFS. Further chest CT suggested multifocal pneumonia within the left lung in addition to apparent cavitary lesions versus bulla, a chronic interstitial lung disease with traction bronchiectasis, calcified right lower lung nodule, and calcified hilar lymph nodes suggesting a history of granulomatosis diseases. A bronchoscopy with Bronchoalveolar lavage was performed. The initial sputum specimen direct smear showed acid-fast stain positive with Actinomyces growth, and Penicillin G was added to the treatment. Samples were sent to the state department lab, and biopsy revealed granulomatous inflammation negative for malignant cells. One month later, the patient's sputum culture showed Tsukamurella for High-performance liquid chromatography (HPLC). Moreover, a rifampicin sensible M. tuberculosis complex by NAA was also positive six weeks later. The patient was started on a complete TB regimen and continued in the outpatient pulmonology clinic with the addition of levofloxacin for three months and rifampicin substituted for rifabutin. As demonstrated in the case above, a Tsukamurella infection can present similarly to a Mycobacterium infection. Patients may be misdiagnosed or potentially be co-infected. Our patient was further tested and appropriately treated for Tsukamurella after further extensive diagnostic screenings. Due to a high rate of missed cases, it is important to keep Tsukamurella infection on the differential diagnosis as the patient presentation may initially appear to be a Mycobacterium or other pulmonary infection. Copyright © 2023 Southern Society for Clinical Investigation.
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SESSION TITLE: Drug-Induced and Associated Critical Care Cases Posters 1 SESSION TYPE: Case Report Posters PRESENTED ON: 10/19/2022 12:45 pm - 01:45 pm INTRODUCTION: Interstitial pneumonitis (ILD) is inflammation of lung interstitium leading to scarring and pulmonary fibrosis. Various etiologies include idiopathic, connective tissue disorders, sarcoidosis and drug induced1. Many chemotherapy agents have been implicated in drug related ILD such as bleomycin, taxanes. However, newer chemotherapeutic drugs such as molecular agents such as anti-VEGF, anti-EGFR (panitumumab) could be causative of drug induced ILD. CASE PRESENTATION: A 75-year-old female with stage IV sigmoid colon cancer treated with surgery, adjuvant FOLFOX chemotherapy and Panitumumab. She presented to the emergency department with shortness of breath and hypoxia after known COVID-19 exposure. Initial imaging with chest radiography showed bilateral ground glass opacities. A chest CT pulmonary embolism protocol was negative for pulmonary embolism but showed bilateral ground glass opacities (GGOs) and some interstitial thickening (L>R) not typical of COVID-19 infection. She was treated with remdesivir and dexamethasone, however her oxygen requirements continued to rapidly escalate. A repeat CT chest without contrast showed bilateral asymmetric interstitial thickening and GGOs. Given persistence of CT chest abnormalities, workup for interstitial lung disease was initiated. The results include ANA titer 1:80, otherwise negative ANCA profile, rheumatoid factor, anti-CCP, Scl-70, Sjogren antibodies. Given clinical history and imaging findings, diagnosis of ILD was suspected, and she was started on solumedrol 1 mg/kg. Her oxygen requirements decreased significantly over the next 2 days, and she was discharged home on oral steroid taper and pneumocystis pneumonia prophylaxis. DISCUSSION: Panitumumab is a fully humanized monoclonal antibody against EGFR. Approved by the US Food and Drug Administration in 2006 for advanced or recurrent colorectal cancer exhibiting wild-type KRAS mutation.2 ILD is rarely reported with panitumumab monotherapy, but higher incidence when used as a combination treatment such as with FOLFOX or FOLFIRI. A Japanese post-marketing surveillance study from 2010-2015 showed an ILD incidence of 1.3% but mortality rates of 51.3%.2 EGFR is expressed on basal cells and non-cilia cells of the bronchioles and type II cells of the alveolus. EGFR mediated mechanisms are important in tissue repair.3 Therefore inhibition of this pathway has been postulated to play a role in development of ILD. Another mechanism was decreased surfactant production by type II cells in pre-clinical study.4,5 ILD secondary to Panitumumab can occur at any point during therapy and up to 1 year after administration of drug.6 The role of infectious processes, in this case, COVID-19 pneumonia, could synergistically worsen ILD presentation. CONCLUSIONS: Although the incidence of ILD is low, the mortality rate is high, therefore early recognition and treatment is associated with improved clinical outcomes. Reference #1: Mudawi D, Heyes K, Hastings R, Rivera-Ortega P, Chaudhuri N. An update on interstitial lung disease. Br J Hosp Med (Lond). Jul 2 2021;82(7):1-14. Reference #2: Osawa M, Kudoh S, Sakai F, et al. Clinical features and risk factors of panitumumab-induced interstitial lung disease: a postmarketing all-case surveillance study. Int J Clin Oncol. Dec 2015;20(6):1063-1071. Reference #3: The FASEB Journal - 2000 - Puddicombe - Involvement of the epidermal growth factor receptor in epithelial repair in asthma.pdf. DISCLOSURES: No relevant relationships by Navitha Ramesh No relevant relationships by Uba Udeh
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SESSION TITLE: Diagnosis of Lung Disease through Pathology Case Posters SESSION TYPE: Case Report Posters PRESENTED ON: 10/19/2022 12:45 pm - 01:45 pm INTRODUCTION: This report describes the case of a patient presenting with pneumothorax and Severe Acute Respiratory Syndrome (SARS) Coronavirus-2 (SARS-cov-2) infection leading to Coronavirus Disease 2019 (COVID-19) pneumonia, with worsening presentation, later found to have underlying Pleuroparenchymal Fibroelastosis (PPFE). CASE PRESENTATION: A 68 year old male with a past medical history of hypertension and type 2 diabetes presented to his primary care clinic with shortness of breath. He underwent a Chest X-Ray as an outpatient which revealed a moderate right-sided pneumothorax (PTX), and he was sent to the Emergency Department by his primary care provider. He was found to be COVID positive on initial workup, also requiring supplemental oxygen. Other routine laboratory tests did not reveal any significant abnormalities. His shortness of breath worsened and on repeat X-rays his pneumothorax increased in size therefore a chest tube was placed by Cardiothoracic Surgery. Computerized Tomography of the chest revealed moderate right pneumothorax, bilateral diffuse ground glass opacities and pulmonary micronodules [Figure 1]. The patient had mild initial improvement and the chest tube was removed but he had recurrence of the PTX and he underwent urgent Video Assisted Thoracoscopic Surgery (VATS), with right upper lobe wedge resection and talc pleurodesis. A biopsy of the resected lung revealed a benign lung with fibroelastotic scarring, diffusely involving subpleural tissue and prominently extending into and entrapping areas of underlying alveolated tissue, with no inflammation, granulomas or pneumonia noted. Workup for tuberculosis, autoimmune disorders, HIV was negative. He eventually was discharged home with close pulmonology and cardiothoracic surgery follow ups, planned for disease surveillance and malignancy workup. DISCUSSION: PPFE is a rare entity, and classified amongst rare causes of idiopathic interstitial pneumonias (IIP) [1]. It is characterized by upper lobe fibrosis, supleural and parenchymal scarring. It can occur at any age, and the usual presentation is of pneumothorax in a thin male, with a shortened anteroposterior diameter of the chest. Radiographic findings typically include subpleural nodular or reticular opacities in the upper lobes, usually sparing the middle and lower lobes. Pathology reveals increased elastic tissue and dense collagen fibers, along with subpleural fibrosis [2]. Pulmonary function testing reveals a restrictive pattern with reduced diffusion capacity and it is usually resistant to steroids [3]. CONCLUSIONS: PPFE is an uncommon cause of insidious, slowly progressive fibrotic lung disease often limited to the upper lobes. It should be suspected in any person presenting with recurrent pneumothorax or blebs without other known inciting causes. Lung biopsy helps establish the diagnosis. Patients with this condition need close pulmonology follow up to assess progression. Reference #1: Travis WD, Costabel U, Hansell DM, King TE Jr, Lynch DA, Nicholson AG, Ryerson CJ, Ryu JH, Selman M, Wells AU, Behr J, Bouros D, Brown KK, Colby TV, Collard HR, Cordeiro CR, Cottin V, Crestani B, Drent M, Dudden RF, Egan J, Flaherty K, Hogaboam C, Inoue Y, Johkoh T, Kim DS, Kitaichi M, Loyd J, Martinez FJ, Myers J, Protzko S, Raghu G, Richeldi L, Sverzellati N, Swigris J, Valeyre D;ATS/ERS Committee on Idiopathic Interstitial Pneumonias. An official American Thoracic Society/European Respiratory Society statement: Update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med. 2013 Sep 15;188(6):733-48. doi: 10.1164/rccm.201308-1483ST. PMID: 24032382;PMCID: PMC5803655. Reference #2: Frankel SK, Cool CD, Lynch DA, Brown KK. Idiopathic pleuroparenchymal fibroelastosis: description of a novel clinicopathologic entity. Chest. 2004 Dec;126(6):2007-13. doi: 10.1378/chest.126.6.2007. PMID: 1559 706. Reference #3: Watanabe K. Pleuroparenchymal Fibroelastosis: Its Clinical Characteristics. Curr Respir Med Rev. 2013 Jun;9(4):299-237. doi: 10.2174/1573398X0904140129125307. PMID: 24578677;PMCID: PMC3933942. DISCLOSURES: No relevant relationships by FNU Amisha No relevant relationships by Perminder Gulani No relevant relationships by Hyomin Lim No relevant relationships by paras malik No relevant relationships by Divya Reddy
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SESSION TITLE: Critical Cardiovascular Disorders SESSION TYPE: Rapid Fire Case Reports PRESENTED ON: 10/18/2022 10:15 am - 11:10 am INTRODUCTION: Physicians are educated on the traditional pathways of sanguineous return of the head and neck through the superior vena cava (SVC). There are known causes of disruption of this system such as SVC syndrome and malignancy causing compression, delayed transit or invasion of these vessels. While compensatory angiogenesis is not a new concept, it has been primarily documented in cases involving coronary artery circulation or congenital heart defects. Here, we present a rare case of the development of left-sided collateral flow in lieu of a right sided SVC with connection to the IVC as a complication of histoplasma infection. CASE PRESENTATION: Our patient was a 67-year female with a past medical history of histoplasmosis, asthma and diabetes who presented with a chief complaint of shortness of breath. Shortly following admission, she was diagnosed with COVID19. In the course of her diagnostic evaluation, she was noted to have significant abnormalities of her thoracic vasculature. More specifically, she had developed calcified granulomas that included a large old calcified granuloma of her right hilum that caused a complete obliteration of her SVC and right middle lobe airways. Her right middle lobe airways had evidence of chronic scarring with development of left sided collateral circulation. Her collateral flow went through her innominate vein into her azygos system and from there into her inferior vena cava and back to her heart. DISCUSSION: It is well established in the literature that histoplasma can lead to scarring and granulomatous changes within lung parenchyma. Our case is unique in the location where the patient developed a granuloma. The close proximity to the SVC over time led to the complete obliteration of the vessel and as a compensatory mechanism her body developed collateral circulation to the left side via her azygous vein and IVC. While we were unable to find similar cases in the literature specifically caused by histoplasma, other phenomena have led to the development of collateral circulation within the lungs. Specifically, Genta et. al. published a case report of an acute pulmonary vein occlusion leading to the development of collateral circulation through the patients' bronchial veins and into the azygous & hemiazygos system similar to our patient. One of the clinical implications for this patient during her hospitalization was the severity of her illness with COVID19. She did require treatment in the intensive care unit. This prompted a discussion among the treatment team regarding developing a plan of action for central line placement should this patient have required vasopressor support. CONCLUSIONS: This case stresses the importance of understanding primary anatomy in order to comprehend potential variants and predict future consequences for patients. It also highlights how sequela of chronic conditions can impact treatment plans. Reference #1: Yu CH, Chen MR. Clinical investigation of systemic-pulmonary collateral arteries. Pediatr Cardiol. 2008 Mar;29(2):334-8. doi: 10.1007/s00246-007-9086-y. Epub 2007 Sep 18. PMID: 17876652. Reference #2: Schaper W. Development of the collateral circulation: History of an idea. Exp Clin Cardiol. 2002 Fall;7(2-3):60-3. PMID: 19649224;PMCID: PMC2719163. Reference #3: Genta PR, Ho N, Beyruti R, Takagaki TY, Terra-Filho M. Pulmonary vein thrombosis after bilobectomy and development of collateral circulation. Thorax. 2003 Jun;58(6):550-1. doi: 10.1136/thorax.58.6.550. PMID: 12775876;PMCID: PMC1746717. DISCLOSURES: No relevant relationships by Alessandra Carrillo No relevant relationships by Chetachi Odelugo No relevant relationships by Shil Punatar No relevant relationships by Ravi Sundaram
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SESSION TITLE: Difficult Diffuse Lung Disease SESSION TYPE: Rapid Fire Case Reports PRESENTED ON: 10/18/2022 10:15 am - 11:10 am INTRODUCTION: Common Variable Immunodeficiency (CVID) is one of the most common humoral immunodeficiency disorders and usually manifests as infectious complications. However, noninfectious complications such Granulomatous-Lymphocytic Interstitial Lung Disease (GLILD) can convey a much poorer prognosis in patients with CVID. In this case report, we present a patient with GLILD who presented with cough and shortness of breath. CASE PRESENTATION: A 66 year old female with past medical history of provoked DVT (off anticoagulation), cervical cancer (s/p resection) presented to pulmonology clinic with complaints of chronic cough and shortness of breath on exertion. She had a negative smoking history and no occupational exposures. She was up to date on vaccinations and COVID was negative. Vitals were stable and physical exam was normal including clear breath sounds. CXR revealed emphysema and hazy opacities in the lung bases. PFTs demonstrated moderate obstructive pattern with no response to bronchodilator, normal lung volumes, and DLCO of 76%. Due to lack of improvement in her cough, CT Chest was done which revealed diffuse pulmonary nodules, bronchiectasis with possible atelectasis or scarring in the RML and lingula, and a prominent subcarinal lymph node. EBUS TBNA of station 7 returned negative for malignancy. Culture showed polymicrobial growth with negative AFB and fungi. Patient was treated without antibiotics, but due to family history of immunodeficiency, immunoglobulin panel was sent which returned low IgG subclasses. She then received IVIG. However, given the centrilobular nodules and lack of response to IVIG, repeat bronchoscopy with TBBx and BAL was performed. BAL revealed lymphocytic predominance and tissue biopsy showed non-caseating granulomas and negative cultures. Eventually patient was diagnosed with GLILD and started on 6 weeks of prednisone 40 mg daily along with PJP prophylaxis. However, her symptoms remained same and rituximab was prescribed with improvement in the symptoms. DISCUSSION: Although recurrent sinopulmonary infections are common in CVID patients, if clinical response to IVIG is minimal to none, GLILD should be considered on the differential. Centrilobular nodules and ground glass opacities should raise suspicion of GLILD and tissue sample should be obtained in these patients to confirm the diagnosis. Appropriate treatment with prednisone or rituximab along with IVIG improves GLILD patient symptoms and yields better outcomes in terms of morbidity and quality of life. CONCLUSIONS: Appropriate treatment with prednisone or rituximab along with IVIG improves GLILD patient symptoms and yields better outcomes in terms of morbidity and quality of life. Reference #1: Hurst JR, Verma N, Lowe D, Baxendale HE, Jolles S, Kelleher P, et al. British lung foundation/United Kingdom primary immunodeficiency network consensus statement on the definition, diagnosis, and management of granulomatous-lymphocytic interstitial lung disease in common variable immunodeficiency disorders. J Allergy Clin Immunol Pract. (2017) 5:938– 45. doi: 10.1016/j.jaip.2017.01.021 DISCLOSURES: No relevant relationships by Benjamin Butler No relevant relationships by Abdulmetin Dursun No relevant relationships by Badri Giri No relevant relationships by Emily Smallwood
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SESSION TITLE: Diagnosis of Lung Disease through Pathology Case Posters SESSION TYPE: Case Report Posters PRESENTED ON: 10/19/2022 12:45 pm - 01:45 pm INTRODUCTION: Usual interstitial pneumonia (UIP) is a histological term used to describe a pattern of interstitial fibrosis with alternating areas of the normal lung with temporal fibrosis and architectural alteration due to chronic scarring or honeycomb change. It is a subset of idiopathic interstitial pneumonias (IPF) that usually presents in the sixth and seventh decades of life with progressive dyspnea on exertion and productive cough. CASE PRESENTATION: We present a 46 y/o man with a history of thyroid disease, hypertension and a former smoker of 20 pack-year smoking. Presented to ED complaining of low oxygen saturation with pulse oximetry at home with readings between 60-80%. Accompanied with progressive dyspnea on exertion and unintentional weight loss of 80 pounds in the last year. Also referred productive cough of white sputum that was worse in the morning. Home nebulized Albuterol therapy did not provide improvement. Denied recent viral respiratory infections, night sweats, environmental exposures nor family history of lung disease. DISCUSSION: Physical exam demonstrated bilateral expiratory dry crackles and pulse oximetry oxygen saturation at room air of 78%. RBBB evidenced on EKG. Bloodwork showed polycythemia with hemoglobin of 17.8;ABG's with pH: 7.40, Pco2: 42.2, PO2: 59.8, HCO3: 26, O2 sat: 90.8 and ideal PO2: 85.6 consistent with metabolic alkalosis with BMP CO2 of 30, A/a gradient: 43.0. Mycoplasma IgM, Influenza A & B and COVID-19 antigen test were negative. CXR with increased vascular markings, chest CT demonstrated small pericardial effusion, bilateral coarse interstitial pulmonary markings and bronchiectasis suggestive of chronic interstitial lung disease with no specific pattern. Left heart catheterization revealed right ventricular hypertrophy, normal EF >55%, and no evidence of coronary disease. Alpha-1 antitrypsin: 158, EPO: 6.5, HIV, and hepatitis panel were all negative. Rheumatology work up with only an ANA antibody positive, with titer 1:160. Patient underwent VATS procedure with wedge biopsy of the right upper and middle lobe that revealed usual interstitial pneumonia pattern. Patient improved and was discharged on home oxygen 3L. At follow-up, treatment was started with Nintedanib and Sildenafil Citrate. He had clinical improvement and oxygen requirements decreased to intermittent oxygen. CONCLUSIONS: Patients with interstitial pulmonary fibrosis experience slow progressive decline with typical clinical presentation over 60 years of age. This case remarks the importance of the need for stratification of interstitial lung disease classification, when pattern and history are non specific, with the use of VATS procedure for early start of treatment. Our patient with no environmental exposure or connective tissue disease had an uncommon early presentation of usual interstitial pneumonia. Reference #1: Tibana, R.C.C., Soares, M.R., Storrer, K.M. et al. Clinical diagnosis of patients subjected to surgical lung biopsy with a probable usual interstitial pneumonia pattern on high-resolution computed tomography. BMC Pulm Med 20, 299 (2020). https://doi.org/10.1186/s12890-020-01339-9 DISCLOSURES: No relevant relationships by Jesse Aleman No relevant relationships by Carlos Martinez Crespí no disclosure submitted for Jean Ramos;No relevant relationships by Alexandra Rodriguez Perez No relevant relationships by Paola Vazquez No relevant relationships by Nahomie Veguilla Rivera
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SESSION TITLE: Post-COVID-19 Infection Complications SESSION TYPE: Case Report Posters PRESENTED ON: 10/17/2022 12:15 pm - 01:15 pm INTRODUCTION: The COVID-19 pandemic remains a burden to healthcare worldwide. Current literature suggests a possible link between COVID-19 survivors and opportunistic infections. We present a case of an immunocompetent male who presented with pneumocystis jirovecii pneumonia (PJP) in the setting of a recent COVID-19 infection. CASE PRESENTATION: A 65-year-old man with 65 pack-year smoking history, COPD, and recent COVID-19 pneumonia requiring hospitalization 1 month prior, presented with 2 days of dyspnea. His physical exam was notable for hypoxia requiring supplemental oxygen and bibasilar crackles. WBC was elevated at 15,500. ABG was significant for hypoxemia. A CT chest demonstrated bilateral peripheral mixed ground glass and consolidative opacities (Figure 1). Upon admission, the patient received ceftriaxone and azithromycin for presumed community acquired pneumonia. However, the patient continued to clinically decompensate with increasing oxygen requirements. As such, a repeat CT was ordered which demonstrated bilateral ground glass opacities, interstitial scarring, and subpleural honeycombing (Figure 2). A bronchoscopy was also performed;bronchoalveolar lavage was positive for PJP by PCR but with negative DFA. The patient was started on trimethoprim/sulfamethoxazole (TMP/SMX) and prednisone. After 3 weeks, the patient clinically improved and was discharged to a skilled nursing facility for rehabilitation. Subsequent CT scan 1 month after initial presentation demonstrated fibrotic changes and bronchial wall thickening (Figure 3). DISCUSSION: In our case, the patient was an immunocompetent male with underlying COPD and recent COVID-19 pneumonia, found to have PJP by PCR. The PCR test for PJP has a higher sensitivity compared to DFA (1), so our patient's incongruent positive PCR and negative DFA test results may represent true PJP or an organizing pneumonia with colonization. A lung biopsy with histology ultimately could have confirmed the diagnosis but was not performed in this case given the patient's clinical improvement with steroids and TMP/SMX (2). Previous studies have demonstrated that COVID-19 can cause immune dysregulation via decreased T cell count and thus can increase the risk for opportunistic infections (3). Furthermore, multiple case reports have shown concurrent COVID-19 and PJP in immunocompetent patients (1). Based on his findings, we believe that our patient was at increased risk for and subsequently developed PJP as a direct consequence of his recent COVID-19 infection. CONCLUSIONS: COVID-19 has been identified as a predisposing factor for subsequent chronic conditions. Studies have demonstrated the capability of COVID-19 infection to weaken the immune system for opportunistic infections as well as remodel the pulmonary architecture. Both conditions can confer high morbidity and mortality for COVID-19 survivors. As such, a close surveillance of this population is warranted. Reference #1: Chong WH, Saha BK, Chopra A. Narrative review of the relationship between COVID-19 and PJP: does it represent coinfection or colonization? Infection (2021) 49:1079–1090. doi:10.1007/s15010-021-01630-9 Reference #2: Culebras M, Loor K, Sansano I, Persiva Ó, Clofent D, Polverino E, Felipe A, Osorio J, Muñoz X, Álvarez A, et al. Histological Findings in Transbronchial Cryobiopsies Obtained From Patients After COVID-19. Chest (2022) 161:647–650. doi:10.1016/j.chest.2021.09.016 Reference #3: Qin C, Zhou L, Hu Z, Zhang S, Yang S, Tao Y, Xie C, Ma K, Shang K, Wang W, et al. Dysregulation of immune response in patients with coronavirus 2019 (COVID-19) in Wuhan, China. Clin Infect Dis (2020) 71:762–768. doi:10.1093/cid/ciaa248 DISCLOSURES: No relevant relationships by Duc Do No relevant relationships by Clara Suh
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Introduction: Definitive chemoradiaotherapy (dCRT) is an option for patients with lung cancer who are medically inoperable or have unresectable locally advanced disease. The local recurrence rate after dCRT is 30% and the prognosis is poor. Salvage surgery, or surgical resection of recurrent disease following dCRT, is one therapeutic option, however, optimal therapy for locoregional recurrences or residual disease is controversial. The purpose of this study was to determine the efficacy of salvage lung resection. Methods: This is a single centre retrospective database review. Patients eligible for the study received definitive chemotherapy, radiation therapy or both followed by salvage pulmonary resection for local recurrence or residual disease. Patient characteristics and outcomes were examined. Results: Sixteen patients (11 male, 5 female) out of 201 that met the inclusion criteria treated between January 2017 and August 2020 were identified with a median follow-up time of 21 months (Q1, Q3 8-37.5). The median patient age was 68. All 16 patients received radiation, 7 of whom received less than 59 Gy and 9 received greater than 59 Gy. The rationale for dCRT varied as 6 patients had disease considered to be unresectable, 5 patients were originally considered to be medically inoperable, 4 patients had a preference for non-surgical management initially, and 1 patient pursued dCRT due to uncertainty of surgical options due to the COVID-19 pandemic. The median time from radiotherapy to surgery was 22 months (Q1, Q3 14.25-27.5). The extent of salvage resections differed as 5 patients had wedge resections, 4 had lobectomies, and 5 patients had more than one lobe resected. No pneumonectomies were preformed. Two resections were aborted in the operating room due to upstaging at the time of resection. The final pathology was 9 adenocarcinomas, 5 squamous cell carcinomas, 1 adenosquamous carcinoma and 1 non-malignant (nodular fibroblastic scarring with surrounding focal organizing pneumonia). Median procedure time was 3h10.5m. Adhesions were noted in 12 cases (75%). Ninety-day mortality was 0%. Overall survival at most recent follow-up was 75% (12 patients). Conclusions: Salvage pulmonary resection after dCRT can be performed with low morbidity and mortality rates and is a good option for treatment of recurrent or residual disease after dCRT. Keywords: Early stage lung cancer treatment, Salvage pulmonary resection, Definitive chemoradiaotherapy
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Introduction: During the pediatric trials for Coronavirus disease 2019 (COVID-19) vaccine the patient population was limited, likely leading to an inappreciable amount of adverse events. As more of the healthy adolescent male population began receiving the COVID-19 vaccination, cases of myocarditis shortly after became more frequently seen. Case Description: A previously well 15-year-old obese male presented to a pediatric ER with 3 days of left arm pain and 1 day of acute left-sided chest pain three days after receiving his second Pfizer-BioNTech COVID-19 vaccine in his left anterior deltoid area. The patient felt unwell afterwards with myalgias, headache, numbness, tingling, emesis, and 1-day history of fever of 38.8°C. He denied feelings of dizziness, syncope, palpitations, change in pain with position or deep breaths. Motrin and Tums did not seem to provide any relief. He had no history of recent viral illness and no known COVID-19 exposure. Initial evaluation included a normal chest Xray and normal sinus rhythm on EKG. Laboratory work revealed elevated troponin-I at 3.18 ng/mL, elevated Total CK at 399 units/L, CK-MB at 19 ng/mL, and BNP <10 pg/mL. Cardiology was consulted and following a normal echocardiogram, the patient was sent for a stat cardiac MRI. The imaging revealed acute myopericarditis with a small pericardial effusion. Mild patchy delayed subepicardial enhancement was also noted in the mid cavity and basal posterolateral wall (suggestive of postinflammatory scarring related to localized myocarditis.) During this time, CK-MB and Troponin-I continued to trend upwards. The patient was then started on standard treatment with Ibuprofen 800 mg Q6H and pantoprazole for gastric protection. His CK-MB peaked at 174 and Troponin-I at 26 which both subsequently trended downwards and normalized prior to discharge. Discussion: Patients who present with chest pain require a broad differential to encompass other possible etiologies including Coxsackie virus, Echovirus, Mycoplasma, EBV, and even Syphilis. Infectious diseases also followed along with the patient throughout his hospital course. All work-up for other potential causes remained negative. 1 week after presentation, his cardiac markers returned to baseline normal values. Conclusion: The study included close to 3,000 adolescents with only 754 ranging in the 16-17 age group further emphasizing the limited power of the study. Myocarditis and pericarditis are known, however rare, side effect of vaccinations and is seen more commonly in males. As the time period between receiving the COVID-19 vaccination and presenting with cardiac symptoms is short it is crucial to provide rapid care and adequate treatment.
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CASE: Patient is a 63 y/o F with PMH of relapsed AML on treatment with Gilteritinib, Meniere's Disease, asthma, GERD, PRA positive, CKD Stage 3. She was on cycle 1 day + 20 of Gilteritinib when she presented with a neutropenic fever of 101.9. She reported congestion and headache. She was pan cultured and started on empiric Cefepime. Her blood cultures, COVID test and CXR were all negative for sources of infection. Eventually, Cefepime was stopped, and she was transited to PO Cefdinir and Cipro but redeveloped fevers and a maculopapular rash. Repeat pan-cultures were negative. Antibiotics were broadened to Merrem, Linezolid and Cresemba and her fevers improved. However, the rash continued to worsen. There was concern that nodular rash was secondary to infection or possible drug reaction from her antibiotics. Her rash showed no improvement with Benadryl or withholding drugs. She underwent skin punch biopsy before discharge. Biopsy showed florid superficial inflammation with benign ulcer that was highly suggestive of Sweet Syndrome given history of AML. IMPACT/DISCUSSION: Sweet syndrome (SS), or acute febrile neutrophilic dermatosis is a rare inflammatory condition characterized by painful cutaneous nodules and neutrophilic infiltrate in the dermis, in the absence of vasculitis. This syndrome is associated with malignancies with AML and MDS being the most reported. Malignancy associated Sweet Syndrome accounts for 15-20% of cases of SS. The atypical production of both pro-inflammatory cytokines (IL - 6, TNF - alpha) and signaling molecules demonstrated in AML is suspected to affect neutrophil function leasing to dermal clumping of the mature neutrophils. In our patient the fever presented prior to the rash with sudden onset of nodular as it has been commonly reported in literature review. Glucocorticoids, either topical or systemic, together with antibiotics and wound care, represent the mainstays of SS therapy. The rash heals without scarring if no ulcerations are present. The signs and symptoms of Sweet syndrome can mimic infection and be treated inaccurately, thus, it is important to make a correct diagnosis. Our patient's tissue cultures were negative for microorganisms. She was started on glucocorticoid with good response in regards to her rash but did have some scars and hyperpigmentation. Unfortunately due to her aggressive AML and complications patient elected to go to Hospice. CONCLUSION: When SS is established, the physician should keep a high index of suspicion to search underlying malignancies. Sweet Syndrome generally responds promptly to treatment with glucocorticoid.
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CASE: We report a 50-year-old Caucasian female with a history of systemic lupus erythematosus (SLE) in remission and chronic kidney disease (CKD) stage 5. The patient presented with dyspnea on exertion and orthopnea for two weeks. Six weeks ago, she was diagnosed with COVID-19 after presenting to the ED for substernal chest pain, myalgias, and fatigue. During this admission, she denied any current joint pain, chest pain, or rashes. She denies a history of alcohol or illicit drug use. EKG in the ED showed T-wave inversions in lead I and aVL, stable from prior EKG. The brain natriuretic peptide level was elevated at 3,500 pg/ml. There was no transaminitis, and kidney function was at baseline. Chest x-ray showed pulmonary vascular congestion and cardiomegaly. A transthoracic echocardiogram showed a left ventricular ejection fraction of 15-20% with severe global hypokinesis. The patient had a full cardiomyopathy workup. We ruled out ischemic cardiomyopathy with a negative coronary angiogram. Non-ischemic cardiomyopathy (NICMO) workup was initiated, with a focus on viral or autoimmune myocarditis. While a cardiac MRI would have been the gold standard to assess for myocardial scarring, the patient's CKD status prohibited this possibility. Similarly, an endomyocardial biopsy was not performed due to its low sensitivity for diagnosing viral or autoimmune myocarditis. Without evidence of infiltrative disease, or other exposures, it was deemed that the patient's recent history of COVID-19 infection, in conjunction with underlying SLE, were the causes of her new-onset NICMO. The patient's dyspnea responded to intravenous bumetanide. We initiated guideline-directed medical therapy with carvedilol and isosorbide-dinitrate. She continues regular follow-up in the outpatient heart failure clinic. IMPACT/DISCUSSION: Classification and evaluation of NICMO can be broad, and thus the clinical picture plays an essential role in the workup. Acquired cardiomyopathy from prior myocarditis was the most likely etiology of our patient's new-onset NICMO. Our patient had no clinical symptoms of myocarditis prior to her exposure to COVID-19, making it unlikely that SLE was the sole driving factor. There is a known association between COVID-19 and myocarditis. A few proposed mechanisms for COVID-19 induced myocarditis include upregulation of cytokines, particularly interleukin-6, and downregulation of ACE2, leading to microvascular and cardiac pericyte dysfunction. Cytokine release from COVID-19 coupled with subclinical SLE could have acted synergistically to cause this patient's condition. Given the increasing incidence of COVID-19 infections, internists must consider COVID-19 exposures during the workup of new-onset heart failure. CONCLUSION: The workup for NICMO in the COVID-19 era must include detailed history taking for sick contacts and prior history of COVID-19 diagnosis. More research is needed to determine if COVID-19 infection can increase the risk of NICMO in patients with a known history of SLE.
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CASE: Our patient is a 52-year-old female with a history of gastroesophageal reflux and hypertension. 36 hours after receiving the second Pfizer COVID-19 vaccine, she developed lip and tongue swelling, mucosal ulcerations, and respiratory distress. There was no conjunctivitis or genital involvement. She denied taking any new medications, supplements, or food that might have led to the reactions. She initially presented to an outside hospital and required intubation prior to transfer to our facility. A bedside esophagogastroduodenoscopy (EGD) was performed noting extensive Grade D erosive esophagitis and gastric ulcerations with friability. When the endoscope was removed a 34cm section of necrotic esophageal tissue was removed from the airway. Despite intravenous steroid treatment, she continued to have esophageal scarring and was unable to tolerate food by mouth. Therefore, a gastrostomy tube was placed. Since that time, she has required several recurrent EGDs for esophageal dilation due to scarring. It has now been six months from her initial injury, and unfortunately, the patient is still unable to take PO and is dependent on tube feedings. IMPACT/DISCUSSION: The coronavirus pandemic began in December 2019. At the time of this report, SARS-CoV-2 infection has been the cause of 5.48 million deaths worldwide and 836,000 deaths in the United States alone. In addition, this global pandemic has had severe economic and social implications. There are currently three vaccines authorized by the United States Food and Drug Administration for emergency use. I report an extremely uncommon complication of the Pfizer COVID-19 vaccine: a case of Eryethema Multiforme Major that occurred after the second dose vaccine without exposure to any other drug. Eryethema Multiforme is divided into major and minor forms and is regarded as distinct from Stevens- Johnson syndrome and toxic epidermal necrolysis. It is related to infections, usually Herpes Simplex Virus, or less commonly, to medications. In Erythema Multiforme, mucous membrane involvement is absent or mild. Erythema Multiforme Major is an immune mediated skin reaction involving the oral cavity and mucosa that is serious and occasionally life threatening. There have been several reported cases of Erythema Multiforme following COVID-19 vaccination but only one other cases of Erythema Multiforme Major associated with the mRnA-1273 SARS-CoV-2 vaccine (Moderna.) CONCLUSION: This case highlights an extremely rare vaccine consequence. The benefits still greatly outweigh the risks of vaccination, and this case does not diminish the importance of COVID-19 vaccination to effectively control this pandemic.