A Critical Care Standpoint in the Diagnosis of Scleroderma Renal Crisis.

Typical or atypical presentations of rare diseases may be confounded by co-morbidities in critically-ill patients. It is imperative to diagnose and treat appropriately, despite this difficulty. Scleroderma renal crisis mimics many other conditions, and can be potentially fatal if not caught early enough. Particularly, in critically-ill patients with multiple pathologies, it can be difficult to distinguish scleroderma renal crisis from other diseases, such as thrombotic thrombocytopenic purpura (TTP), hypertensive emergency, posterior reversible encephalopathy syndrome (PRES), or atypical hemolytic uremic syndrome (HUS). Herein, a patient who presented with encephalopathy and seizures was initially treated for thrombotic thrombocytopenic purpura, but was ultimately diagnosed with scleroderma renal crisis. Given her numerous laboratory abnormalities, such as thrombocytopenia, hemolytic anemia, kidney and liver dysfunction, and elevated inflammatory markers, various differentials were considered. During her hospitalization, she suffered a cardiac arrest, seizures, nosocomial infections and worsening kidney disease requiring dialysis, making the final diagnosis of scleroderma renal crisis a diagnosis of exclusion. Subsequently, the management of a patient with multiple co-morbidities and confounding laboratory abnormalities difficult to treat. This article highlights these intricacies and formulates the thought process behind the diagnosis of Scleroderma Renal Crisis.

Panniculitis and pancreatitis: Inflammation and necrotic mechanisms in a patient with alcohol use disorder and alarming suspicion for malignant process.

Panniculitis is an inflammatory process localized to subcutaneous tissue, with etiologies including infection, malignancy, external insults, enzymatic destructive processes, and inflammatory disorders. The incidence of panniculitis manifesting as necrosis of subcutaneous fat tissue associated with pancreatic diseases is low, which may encompass associated periarthritis with bone necrosis and panniculitis (Pancreatitis, panniculitis and polyarthritis syndrome). Pancreatitis, panniculitis and polyarthritis syndrome is considered to derive from the systemic activity of enzymes within the pancreas, which leads to disturbances within the microcirculatory system, and fat necrosis of medullary bone marrow; however, the exact pathophysiology remains unknown. Here, we present a case of a 53-year-old male with a history of chronic pancreatitis who presented with lower abdominal pain found to have osteolytic pelvic lesions considered to be panniculitis secondary to pancreatitis. Our patient provided an interesting clinical picture given his alcohol use disorder, and lytic lesions which lead the team initially towards a malignant etiology of panniculitis such as myeloma; however, given his negative studies, it was presumed his panniculitis was derived from his chronic pancreatitis. Overall, additional literature is warranted regarding the extensive workup of lytic bone lesions that present in patients who have acute vs chronic pancreatitis.

Rare Isolated Duodenal Hamartomatous Polyp in an Elderly Patient.

BACKGROUND Hamartomatous polyps represent rare sporadic lesions, characterized by fibrous stroma, vascular infiltration, and dilation of mucous glands. The lesions present in a bimodal fashion in adults as well as children from 1 to 7 years old, and are often diagnosed during endoscopic procedures. Specifically, solitary Peutz-Jeghers represents a type of hamartoma that has similar histologic features to typical Peutz-Jeghers syndrome. Hamartomatous polyps represent disorganized tissue growth and can bear relationships with genetic syndromes classified as hamartomatous polyposis syndromes. A number of these syndromes, such as Peutz-Jeghers and Cowden syndrome, can demonstrate an increased risk of malignancy. A variety of symptoms, or no symptoms at all, can accompany these polyps, such as abdominal discomfort, bowel obstruction, gastrointestinal bleeding, or intussusception in severe cases. Histologically, these polyps appear similar to Peutz-Jeghers syndrome growths; however, they lack extraintestinal manifestations. Given fairly low risk of development into malignancy, patients have a good prognosis if presenting with a solitary hamartomatous polyp. There is limited data regarding screening guidelines for this patient population. CASE REPORT Here, we present a rare case of a 73-year-old woman who had a history of anemia and status post endoscopic evaluation and was diagnosed with a benign hamartomatous polyp (juvenile-like), histologically consistent with tubulovillous adenoma. CONCLUSIONS Differentiating sporadic polyps from syndromic polyps is important, as sporadic polyps have a benign course, while those associated with a syndrome have an increased lifetime malignancy risk.

A rare case of a peripheral Ewing sarcoma primitive neuroectodermal tumor of pelvic origin.

Primitive neuroectodermal tumors (PNET) represent malignant neuroectodermal tumors composed of small round cells. They can be differentiated between originating from the peripheral nervous system or the central nervous system. Peripheral PNET (pPNET) can be further subclassified as one of the Ewing family tumors (EFT). Although rare, EFT can originate in the female genital tract and pelvic region. Here, we present a case of a middle-aged female with PNET masses in her uterus, abdomen, and hepatic lobes. We discuss the diagnostic modalities, including immunohistochemistry, histopathology, and imaging findings associated with this rare malignancy.

Aleukemic Acute Promyelocytic Leukemia: How Concomitant HIV, Hepatitis C, and Chronic Alcohol Use Disorder May Have Hidden an Underlying Malignancy.

BACKGROUND Acute promyelocytic leukemia (APL) is a rare subtype of acute myeloid leukemia (AML) and is characterized by a genetic translocation affecting the retinoic acid receptor-alpha gene, leading to blockage in the differentiation of granulocytic cells. The accumulation of promyelocytes in bone marrow leads to cytopenias and life-threatening coagulopathies. Definitive diagnosis is made with bone marrow biopsy. Differentiation of APL from other leukemias is important to appropriately treat with all-trans retinoic acid (ATRA) and arsenic trioxide. Patients with HIV are at a higher risk to develop AML. This article identifies how multiple comorbidities and social factors can contribute to difficulties in diagnosing AML. CASE REPORT We present a 67-year-old man with a past medical history of hypertension and substance use disorder who presented with progressive exertional dyspnea and was found to have HIV, chronic hepatitis C, and APL with pancytopenia. His bone marrow biopsy confirmed AML. This was a case of co-existing HIV and aleukemic leukemia. CONCLUSIONS APL can present with pancytopenia, weakness, failure to thrive, or bleeding complications, which can be similar to presentations of those diagnosed with HIV. Diagnosis of APL can be differentiated between hypergranular and hypogranular; our patient demonstrated APL with only 52% blasts, which can make for a challenging diagnosis. Given increased mortality of APL, immediate ATRA therapy is warranted. Aleukemic leukemia is a rare presentation typically accompanied by skin manifestations. Our case highlights the importance of having high clinical suspicion for malignancy in patients with comorbidities that can interfere with the classic presentation of leukemia.

A Challenging Situation: Empirical Treatment with Therapeutic Plasma Exchange in a Patient with Sickle Cell Disease.

Sickle Cell Disease (SSD) can present with acute painful crises, most commonly manifesting as diffuse bony pain; however, rare presentations of acute coronary syndrome, acute papillary necrosis, or multi-organ failure may also present in these patients. TTP has been rarely described in conjunction with sickle cell pain crisis (SS crisis). In both TTP and sickle cell crises, widespread platelet activation is present with thrombocytopenia as a result. Thrombocytopenia can be utilized as a poor prognostic indicator in patients with SS crisis. Multi-organ failure may appear similar to TTP and patients may benefit from similar therapy. Here, we present a 27-year-old female with a history of SSD who presented with a painful crisis who was found to have worsening renal failure and thrombocytopenia and was treated empirically with therapeutic plasma exchange (TPE), later discovered to have SS crisis with multi-organ failure with unremarkable ADAMSTS13 values. Given the high fatality risk of TTP, the benefits outweighed the risks for empiric TPE therapy, and our patient benefited from the treatment, as patients with both TTP and/or SS crisis multi-organ failure have demonstrated improvement following this treatment. Given the severity of multi-organ failure in SSD patients, additional research is warranted for improvement in the diagnosis and management of these patients.

Rare Case of Refractory Hypokalemia in a Patient with Acute Monocytic Leukemia.

BACKGROUND Refractory hypokalemia has been rarely demonstrated in patients with acute monocytic leukemia (AMoL). Hypokalemia develops in these patients owing to renal tubular dysfunction, secondary to lysozyme enzymes that are released by monocytes in AMoL. Additionally, renin-like substances are produced from monocytes and can lead to hypokalemia and metabolic alkalosis. There is also an entity called spurious hypokalemia, in which high numbers of metabolically active cells in blood samples increase sodium-potassium ATPase activity, resulting in influx of potassium. Additional research is warranted regarding this specific demographic to create standardized treatment approaches to electrolyte repletion. CASE REPORT In this case report, we demonstrate a rare case of an 82-year-old woman with AMoL, complicated by refractory hypokalemia, who presented with concerns of fatigue. The patient's initial laboratory results were significant for leukocytosis with monocytosis and severe hypokalemia. Refractory hypokalemia was noted, despite administration of aggressive repletions. During her hospitalization, AMoL was diagnosed and an extensive workup was performed to evaluate the underlying cause of hypokalemia. Ultimately, the patient died on day 4 of hospitalization. We describe the correlation between severe refractory hypokalemia and leukocytosis and provide a literature review of multiple etiologies of refractory hypokalemia in patients with AMoL. CONCLUSIONS We evaluated the numerous pathophysiologic mechanisms responsible for refractory hypokalemia in patients with AMoL. Our therapeutic outcomes were limited owing to the patient's early death. It is of high importance to evaluate the underlying cause of hypokalemia in these patients and to treat accordingly with caution.

Hepatitis of an Unknown Etiology with Concomitant ITP.

Alcoholic hepatitis (AH) usually presents after decades of alcohol consumption and can even manifest with recent abstinence. The clinical presentation may be compounded by underlying liver cirrhosis and liver function enzymes are not a reliable means of diagnosing AH due to poor sensitivity and specificity. One feature of alcoholic hepatitis is thrombocytopenia; however, patients may also have thrombocytopenia due to another underlying condition, such as Immune Thrombocytopenic Purpura (ITP). ITP is an autoimmune disease caused by autoantibodies against platelet glycoproteins. ITP is a diagnosis of exclusion and secondary causes of thrombocytopenia must be ruled out with persistent thrombocytopenia that is refractory to treatment for AH. Although there is limited data demonstrating a correlation between AH and ITP, both conditions respond to steroids. We present a case of a 42 YO M with an unknown cause of hepatitis and concomitant ITP who responded well to steroids.

Autoimmune hepatitis presenting with concomitant chronic pancreatitis.

Autoimmune Hepatitis (AIH) is a progressive form of chronic hepatitis, with periods of remissions and exacerbations. Diagnosis includes abnormally high levels of immunoglobulins and multiple autoantibodies. Clinical presentation is variable, with a spectrum extending from asymptomatic cases to fulminant liver failure. Symptoms include abdominal pain, malaise, fatigue, and small joint arthralgia. We present a case of a 36-year-old male with a past medical history of alcohol dependence and acute pancreatitis who was diagnosed with AIH. There is limited data regarding patients with concomitant AIH and pancreatitis. Our patient presented with AIH with secondary acute on chronic pancreatitis, in the absence of additional autoimmune manifestations. The mechanism of AIH remains poorly understood; however, there is an association between the HLA gene and AIH. Genetic studies have shown HLA-DRB1*0301 and HLA-DRB1*0401 as primary and secondary genotypes susceptible to AIH, as well as genetic variants with CARD10 and SH2B3. Products secondary to metabolism of ETOH such as alcohol dehydrogenase, malondialdehyde, and acetaldehyde, can lead to development of autoantibodies. Additional research is indicated to evaluate the relationship between AIH and acute pancreatitis.

Initially Suspected Anaphylaxis Following Iodinated Contrast: Jod-Basedow Phenomenon in a 73-Year-Old Female Without a History of Thyroid Dysfunction.

The Jod-Basedow phenomenon (JB phenomenon), also referred to as "iodine-induced hyperthyroidism," rarely occurs. Radiological imaging using iodinated contrast contains a dose of 300 to 1221 mg of iodine per kilogram, which can transiently induce clinically significant hyperthyroidism (referred to as Jod-Basedow Syndrome) in euthyroid patients. Hence, the reporting of such events is important for clinicians to be aware of, to prevent unnecessary iodine-based imaging. Underlying thyroid abnormalities, including latent Graves' disease, autoimmune thyroiditis, use of iodine-containing foods or medications, such as amiodarone, and Lugol's iodine have been shown to increase the risk of JB phenomenon. In terms of the pathophysiology of the JB phenomenon, when iodine exposure is in excess, increased iodine leads to increased hormone synthesis, and with an absence of auto-regulation, this can lead to thyrotoxicosis. In this case report, we describe the iodine-induced JB phenomenon in a 73-year-old female with no prior thyroid dysfunction, who was initially admitted for pyelonephritis and was eventually transferred to the intensive care unit secondary to suspected anaphylaxis.

The unpredictability of labile blood pressure: Afferent baroreflex failure in a critical patient with multiple thyroid surgeries and COVID-19 infection.

The carotid sinus-arterial baroreflex is essential in maintaining blood pressure (BP) regulation. Afferent baroreflex failure (ABF) can present with labile changes in BP within seconds and can be secondary to neck surgery or radiation. We present here the first case, to our knowledge, of ABF precipitated by thyroidectomy, in a patient with active COVID-19 pneumonia, causing difficult control of severely labile BP in a critical care unit. Contributing factors included her critical illness state with upregulation of IL-6 leading to pituitary-adrenal axis alteration, her thyroidectomy further exacerbating autonomic dysfunction, as well as downregulation of ACE2 via COVID-19 infection. Management was achieved with a combination of midodrine and clonidine catered to specific BP thresholds. Additional research with a multidisciplinary approach is warranted to fully optimize the treatment of ABF in patients with neck surgery and or inflammatory conditions such as COVID-19.

COVID-19 Viral Infection Presenting With Diabetic Ketoacidosis, Hyperosmolar Hyperglycemic Syndrome, or Mixed Hyperglycemic Crisis: A Case Series.

Cardiovascular disease, COPD, and diabetes (DM) are associated with increased complications with COVID-19. A correlation between COVID-19 and diabetic ketoacidosis (DKA) or Hyperosmolar Hyperglycemic Syndrome (HHS) has been suggested; however, the precise mechanism remains unclear. We present a case series of six patients with COVID-19 infections who were found to have DKA, HHS, or mixed picture. Wedescribe an association between COVID-19 and hyperglycemic emergencies. Six patients (50% male, 50% female, mean age 47.667 ± 18.747) were identified from November 2021 to February 2022. Comorbidities included DM (83.3%), HTN (50%), as well as ESRD, A-Fib, ISLD, HIV, and dementia (each 16.7%). Common review of systems included nausea and vomiting (50%), abdominal pain (33.3%), dyspnea (33.3%), and decreased appetite (33.3%). Additional findings were dysarthria, facial droop, generalized weakness, productive cough, myalgias, and increased urinary frequency (16.7%). Patients were diagnosed with DKA (50%), mixed process (33.3%), andHHS(16.7%). In terms of COVID-19 symptoms, most patients were asymptomatic (83.3%), with one patient developing hypoxia. The survival rate was 100%. Infections can incite DKA/HHS; yet, COVID-19 may have factors that amplify this process, in the setting of pancreatic beta-cell dysfunction from the virus itself. This may contribute to why diabetic patients have a ten times higher risk of death if they develop COVID-19. This virus binds to ACE2 receptors in the pancreas and damages the islets, ultimately decreasing insulin release. Here, we introduce cases of DKA/HHS in the setting of COVID-19, to understand the relationship between how COVID-19 infections may exacerbate diabetic complications.

Metronidazole induced cerebellar toxicity after prolonged treatment of large multiloculated pyogenic liver abscess; A case report and literature review.

Metronidazole is a common antibiotic agent for hepatic abscesses, which require both gram-negative and anaerobic coverage. Rarely, this antibiotic has been found to induce encephalopathy. Here, we describe a 65-year-old male who was treated with metronidazole for his hepatic abscess, who presented with syncope and questionable seizure and was found to have magnetic resonance imaging (MRI) brain findings consistent with metronidazole toxicity. Our patient demonstrated striking brain MRI findings which can be used to further understand the process behind this medication-induced toxicity. Hypotheses of this mechanism include swelling of axons secondary to increased water or vasospasm leading to reversible ischemia that is localized in the brain. In terms of MRI findings, brain lesions tend to populate bilaterally with focus at the dorsal pons, midbrain, cerebellar dentate nuclei (as with our patient), dorsal medulla, or splenium of corpus callosum. Additional research is warranted regarding this rare manifestation and timely removal of the offending agent is crucial for reversal of symptoms.

Carcinoma Erysipeloides: An Underdiagnosed Phenomenon of Cutaneous Metastases of Breast Cancer.

BACKGROUND Cutaneous metastatic breast cancer is a rare manifestation. Causes include vascular or lymphatic spread or iatrogenic mechanisms following surgery. A sub-type of this disease process, "Carcinoma Erysipeloides," represents subcutaneous and dermal tissue layer invasion via lymphatic spread. Diagnosis can be challenging, and therefore, obtaining a thorough history and physical, with careful inspection of prior surgical scars is essential for an accurate diagnosis. Lesions present in variable ways, including papules, plaques, ulcerations, nodules, crusting, or fungating masses, with common locations in the chest, scalp, abdomen, and less commonly the arms. When carcinoma erysipeloides is identified, it is imperative to evaluate for distant metastases. Recent literature has identified benefits with trastuzumab deruxtecan therapy instead of trastuzumab emtansine, with decreased progression rates and decreased mortality rates. Metastasis to the skin can indicate advanced disease; however, this metastatic site may be preferable to visceral organs or bones in terms of prognosis. CASE REPORT We present a rare manifestation of metastatic breast cancer in 45-year-old Hispanic woman, status post neoadjuvant chemotherapy and radical cystectomy on maintenance trastuzumab and pertuzumab. We discuss the clinical presentation variability, keys to diagnosis, treatment considerations, and outcomes for this unique patient population. CONCLUSIONS Carcinoma erysipeloides varies in clinical presentation, especially when patients develop exclusive skin lesions. We identify common etiologies for this progression of disease and discuss combination therapy which has demonstrated a reduction in mortality in this patient population.

A rare case of metastatic pancreatic adenocarcinoma presenting as a pulmonary embolism from nonbacterial thrombotic endocarditis.

Nonbacterial thrombotic endocarditis (NBTE) also called, "Marantic endocarditis" occurs due to an underlying hypercoagulable state causing tissue damage and upregulation of the coagulation cascade, with noninfective vegetation formation on heart valves. Mitral and aortic valves are most commonly involved. NBTE is rare, with an incidence of 1.6%, with 65 cases identified during a 10-year autopsy analysis. The most common malignancies associated with NBTE include gynecological cancers, lung cancer, gastric cancer, and pancreatic cancers with adenocarcinoma histology being the greatest risk. Herein, we present a rare case of a 55-year-old male who presented with acute hypoxic respiratory failure secondary to pulmonary embolism due to nonbacterial thrombotic endocarditis. He was found to have advanced pancreatic adenocarcinoma on further investigation of the 2.2 cm hypodense cystic mass in the distal pancreatic body and tail, and complex liver masses which were incidentally found on computed tomography angiography (CTA) of the chest. This is a rare phenomenon and clinicians have to consider the hypercoagulable state associated with cancers, particularly pancreatic adenocarcinoma, and the risk of NBTE.

Recurrent Hypokalemia as an Unusual Presentation for Metastatic Adrenocortical Cancer.

Adrenocortical cancer is a rare malignancy of the endocrine system. Therefore, when this malignancy presents with metastatic disease, this leads to further difficulties in management. Due to the rare and ambiguous nature of this malignancy, diagnosis is generally made at later stages, with limited options for patients. Symptoms may include weight gain/loss, muscle weakness, abdominal discomfort/bloating, hyperglycemia, hypertension, electrolyte imbalance, hirsutism and virilization in females, gynecomastia and hypogonadism in males. Due to the variety of conditions presenting with one or more of these symptoms, diagnosis can be difficult. Many adrenocortical tumors, malignant and benign, are usually detected incidentally on imaging performed for evaluation of another condition, also known as "incidentalomas." Here, we present a rare case of metastatic adrenocortical malignancy in a 56-year-old female patient who presented with isolated recurrent episodes of hypokalemia.

Aortic dissection presenting as stroke with focal neurologic deficits: A case report.

Aortic dissection is a relatively uncommon and potentially misdiagnosed disease. Early and accurate diagnosis and appropriate treatment are imperative for patient survival. In this case, we present a 55-year-old male who presented to the Emergency Department with severe abdominal pain and acute neurological deficits who was triaged for stroke protocol. After unexpected findings on physical examination, other diagnostic hypotheses were evaluated, culminating in the diagnosis of aortic dissection. The purpose of this article is to exemplify a patient who presents with noteworthy neurologic clinical features who ultimately was diagnosed with Type B aortic dissection, to help benefit the rapid diagnosis and subsequent treatment for future patients with similar presentations.

Gynecomastia and Malignancy: A Case of Male Invasive Ductal Breast Carcinoma Treated with Neoadjuvant Chemotherapy.

BACKGROUND Male breast cancer represents a rare malignancy with identifiable risk factors, including genetics, radiation exposure, liver dysfunction, and concomitant diagnosis of Klinefelter syndrome. Gynecomastia can commonly present in these patients, and despite increased estrogen levels in adipose breast tissue, gynecomastia has not been proven to be a significant risk factor for carcinoma development. Male patients with new-onset breast masses are recommended to undergo diagnostic mammograms and breast ultrasound for further evaluation. Those diagnosed with breast cancer most commonly have invasive ductal carcinoma of the breast, and over half of these patients are found to have estrogen and progesterone receptor (ER/PR) positivity. CASE REPORT In this case report, we present a Black man with gynecomastia and an areolar lesion for a 6-month duration following a traumatic event. He was initially referred to the surgical team for further evaluation, and subsequent imaging and biopsy data revealed ER/PR-positive invasive ductal carcinoma. Multidisciplinary discussions were held, and the patient was arranged to begin neoadjuvant treatment with doxorubicin hydrochloride and cyclophosphamide, followed by treatment with paclitaxel (AC-T) chemotherapy, followed by bilateral mastectomy and adjuvant hormonal therapy. CONCLUSIONS The treatment of male breast cancer has remained relatively like that of female breast cancer, which may be due to the limited data in the treatment of male breast cancer. Thus far, studies involving neoadjuvant chemotherapy of female patients have demonstrated promising responses to expand surgical options for patients and possibly decrease the rates of recurrence. Additional studies are warranted to discern optimal therapy for the male patient population.

Cerebral venous sinus thrombosis in pregnancy presenting with hemiplegia: A case report.

Cerebral venous sinus thrombosis (CVST) is a rather uncommon disorder. CVST is potentially lethal, therefore early detection and treatment is critical. CVST has been linked to pregnancy and puerperium, while COVID-19 infection has been linked to a hypercoagulable state. CVST can be difficult to detect and treat due to the wide range of neurological manifestations, especially in patients with hypercoagulability. The goal of this study is to conduct a literature review and present a unique case of a pregnant woman with CVST who had left hemiplegia and headache. After 6 months of treatment in the hospital, the patient's hemiplegia was fully resolved. Here, we discuss the treatment of CVST in pregnant women who have a suspected past COVID-19 infection.

An Unexpected Transformation: Malignant Spindle Cell Carcinoma Developed From Primary Basal Cell Carcinoma.

Spindle cell carcinoma (SpCC)/sarcomatoid carcinoma is a biphasic tumor with molecular and histopathological properties of both epithelial and mesenchymal tumors. SpCC usually occurs either in sun-exposed areas like the head, neck, upper extremities, and chest or in the areas of skin with prior radiation exposure or in immuno-suppressed individuals. Cutaneous SpCC is a very rare disease, with only a handful of reported cases so far. SpCC differs from conventional squamous cell carcinoma (SCC) with dermal infiltration of atypical keratinocytes as single cells with hyperchromatic eosinophilic cytoplasm and elongated, pleomorphic nuclei with multiple nucleoli, in contrast to cohesive nests or islands in SCC. The objective of this study is to complete a review of the current literature and present a rare manifestation of malignant SpCC which developed from a localized basal cell carcinoma following excision and radiation therapy (RT) in a 79-year-old female. We plan to elucidate the importance of a timely and accurate diagnosis of this disease in order to maximize treatment options and improve survival outcomes.