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2023 AMA Research Challenge

A Case of Dual Left Anterior Descending Coronary Artery Supply with a Myocardial Bridge

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Introduction

Atrial fibrillation is the most common arrhythmia in the world. Deglutition-induced tachyarrhythmias are rare, composing only 0.6% of the cases. These arrythmias occur during swallowing due to esophageal stimulation, likely involving vasovagal and adrenergic reflexes. Diagnosis relies on a documented history, Holter monitoring and echocardiograms. Lifestyle changes, medications including beta-blockers and antiarrhythmics, and radiofrequency catheter ablation are have shown to be viable treatment options. 

Case Presentation
A 58-year-old Caucasian woman presented at the emergency department with frequent palpitations. Her medical history included esophageal strictures with recent esophageal dilation, chronic immunosuppression post-kidney transplant, hypertension, hyperlipidemia, and hypothyroidism.

Prior to symptoms, patient underwent an esophageal dilation at an outside hospital. Following the procedure, she experienced short-lived, episodes of palpitations upon swallowing. At presentation, patient had an unremarkable physical examination. Labs showed a normal thyroid-stimulating hormone level, mild hyperbilirubinemia, and mild leukocytosis. The electrocardiogram showed normal sinus rhythm with minimal ST segment abnormalities and normal QTc. A Holter monitor was placed to further investigate the palpitations, The results showed sinus rhythm and mild sinus bradycardia with episodes suggestive of atypical atrial fibrillation. It was consistently noted these episodes were triggered by swallowing, leading to a diagnosis of deglutition-induced atrial fibrillation. The patient was prescribed metoprolol 25mg twice daily. The rate-controlling agent was switched from metoprolol to propafenone 225mg twice daily due to failure to eliminate the palpitations. Ultimately due to side effects of dizziness from the propafenone, the regiment was changed to flecainide 75mg twice daily. In subsequent follow-ups, the patient noted complete resolution of her symptoms.

Discussion
Swallowing-induced supraventricular tachycardias, like deglutition-induced atrial fibrillation, are exceptionally rare, representing less than 1% of reported atrial fibrillation cases with under 100 documented instances. The mechanisms underlying these tachycardias remain uncertain, with leading hypotheses suggesting mechanical left atrial stimulation after esophageal distention or activation of the vagus nerve due to increased intra-esophageal pressure. While first line treatment options involve beta-blockers, they have shown limited effectiveness, resolving symptoms in only about 18% of cases. Propafenone has shown promise, terminating supraventricular tachyarrhythmias in approximately 83% of cases, including deglutition-induced atrial fibrillation. Other antiarrhythmic medications like digoxin, flecainide, and procainamide have shown success in some cases. Radiofrequency ablation is a permanent therapeutic option with nearly 100% success rate for cases where pharmacological treatments are insufficient. Due to the rarity of deglutition-induced atrial fibrillation, extensive research is necessary to understand its mechanisms fully and optimize treatment strategies.

An Exceedingly Rare Case of Deglutition-Induced Atrial Fibrillation

Introduction:

Mycotic aneurysms are a rare complication of Infective Endocarditis (IE). They can be the result of septic emboli from the heart occluding the vasa vasorum of the vessel or the vessel lumen leading to vascular wall infection. Though mycotic aneurysms can develop anywhere, they are most commonly seen in the intracranial arteries, followed by visceral, then upper and lower extremities arteries at the arterial bifurcations. In a review of 922 of definite IE, symptomatic mycotic aneurysms were observed in 2 percent of cases. Of these, 66% were intracranial (especially involving MCA), as in our patient. CT Angiogram (CTA) is the most useful imaging to diagnose infected aneurysms.

Case Presentation:

We present the case of a 45-year-old African American male who presented with encephalopathy and left hemiparesis and was found to have a right inferior MCA infarct, along with evidence of lucency in the right posterior insula, posterior frontal lobe, and anterior parietal lobe. On Transthoracic Echo (TTE), was found to have friable vegetation on the Mitral, Aortic, and Tricuspid Valves; also, was found to have an infection of the aorto-mitral curtain. On CTA chest and CTA head/neck, was also found to have Aortic root abscess, Aortic to Right Ventricle fistula, diffuse pulmonary opacities, and 2 mycotic saccular aneurysms on right M2 MCA measuring 6 x 5 mm and 4 x 4 mm. Given that the patient grew Enterococcus Faecalis in his blood cultures, management was treatment with Rocephin and Ampicillin for which E-faecalis was susceptible to. Neurology and Cardiothoracic (CT) surgery were also consulted. Hospitalization was further complicated by ruptured mycotic aneurysms resulting in Subarachnoid and Subdural Hematomas. Given the friability of the valves and brain bleed, he was deemed not a surgical candidate and ultimately decided on palliative measures given his poor prognosis.

Discussion:

This case presents a rare complication of disseminated infective endocarditis such as mycotic aneurysms. There is no true consensus in management, however, the current standard of treatment is antibiotic therapy with consideration of surgical debridement with or without revascularization. In one study of 26 patients studied retrospectively, 60% of them had ruptured aneurysms, though only 5 patients required clipping of aneurysm and 8 patients had intraparenchymal hemorrhage (1). In another case series of 25 patients with mycotic aneurysm, 10 patients had these due to IE, and 5 patients had early surgery but no case of emergent surgical procedure for decompression or aneurysmal repair were required in these patients (3). Therefore, a review of literature revealed rupture rate can vary from <2% to 72% (4).

Conclusion:

Our case demonstrates the importance of screening for vascular complications in the post-stroke workup for any patient who presents with a stroke in the setting of IE. Additionally given the wide variation of aneurysmal rupture, early antibiotic treatment remains the mainstay of management, though early surgical evaluation and debridement should be considered.

Severe case of disseminated infective endocarditis

Introduction
Colon cancer is one of the top five leading causes of cancer in both males and females. There is a well-known association between colon cancer and microbial infections. The presence of certain bacterial infections in the appropriate clinical context should prompt further investigation of colon cancer.

Case Presentation
A 41-year-old male with history of alcohol use disorder and bipolar disorder who presented for acute encephalopathy after cardiac arrest with return of spontaneous circulation within 2 minutes in the field.
In the emergency department he was found to be hypotensive and hypoxic; labs revealed leukocytosis (50.9 K/ul) with bandemia (15%), anemia (5.9g/dl), and LFTs consistent with acute liver failure. CT abdomen/pelvis showed hepatosplenomegaly, pelvic ascites, colonic wall thickening and luminal narrowing in mid-sigmoid colon.
He was admitted to the ICU for twelve days for septic shock intermittently requiring vasopressors, acute liver failure, and respiratory failure. A diagnostic paracentesis revealed a transudative effusion; blood cultures were positive for Clostridium septicum and Enterococcus avium.
A subsequent colonoscopy demonstrated a sigmoid colonic mass whose pathology was consistent with adenocarcinoma. Due to his acute decompensated liver failure secondary to alcohol use; he was not a candidate for surgery. The family placed the patient in hospice care and he eventually expired.

Discussion
Colon malignancy has been associated to certain bacterial strains, with the most common being Streptococcus bovis/gallolyticus, and less frequently Clostridium septicum, Enterococci species, Bacteroides fragilis, Fusobacterium nucleatum, and other Streptococci species. In fact, of the approximately 106,180 new cases of colon cancer reported yearly, 10-25% are associated with infections caused by Streptococcus bovis and Clostridium septicum.
Clostridium septicum is an opportunistic, gram-positive anaerobe that has several virulence factors, including alpha toxin. It can survive in acidic environments due to its ability to produce spores and has the capability to perforate the colonic epithelial lining, releasing spores into the blood and leading to bacteremia. The risk factors for C. septicum infection include immunosuppressed states, hematological malignancies, neutropenia, and diabetes mellitus. Unfortunately, C. septicum infection has a high mortality rate of 60%, which can only be diagnosed through positive cultures.
Treatment for C. Septicum infection varies depending on the presentation, as it can cause aortitis, myonecrosis, gangrene, cellulitis, septic shock, fasciitis, hemolysis, and abscess. However, early antibiotics and possible surgical intervention are recommended for effective management. Piperacillin/tazobactam or penicillin G are the first-line treatments for C. septicum infection. Therefore, it is crucial to identify and manage this infection as early as possible to prevent the high mortality rate associated with it.
Conclusion
Clostridium septicum infection and colonic malignancy is a well-known association therefore clinicians should have a high suspicion for colon cancer in patients who present with this infection.

A Potentially Lethal Harbinger: Clostridium Septicum Bacteremia in a Patient Found to have Adenocarcinoma of the Colon

Background: 
Monkeypox, caused by the Monkeypox virus (MPV), is a double-stranded DNA virus belonging to the genus Orthopoxvirus. While confirmed cases of Mpox disease have been reported in since the 1970s, the recent global outbreak has escalated Monkeypox to a public health emergency. Although Monkeypox is typically considered a self-limiting disease, the recent outbreak has demonstrated an increase in disease morbidity and mortality, particularly in those with immunocompromised status. For those with HIV infection, co-infection with Monkeypox can lead to atypical disease presentation. This poster highlights an example of a highly atypical dermatological presentation in a patient with HIV and Monkeypox co-infection. 

Discussion: A 28-year-old male with a history of untreated HIV/AIDS presented with diffuse bilateral eschars with well-extended surrounding erythema for two days. The lesions were expansive and located diffusely across the bilateral upper extremities. The patient reported the lesions were slowly enlarging and mildly pruritic. Additionally, bilateral tender inguinal lymphadenopathy and a single painful shallow ulcer on the right-side penile shaft was present. While Monkeypox typically presents with small well-localized eschars surrounded by slight erythema, this patient demonstrated well-extended erythema and diffuse eschars on the bilateral upper extremities. This case illustrates that HIV and Monkeypox co-infection may lead to exacerbation of the typical symptoms of each disease– resulting in atypical dermatologic presentations, unique disease manifestations, and more difficult management. 


Rare presentation of Monkeypox in a Patient with Coexisting HIV

Background
Scleroderma is a rare, chronic autoimmune disease that is characterized by thickening of the skin and other connective tissues in the body. Nodular scleroderma (NS) is a rare cutaneous form of scleroderma that can occur primarily with systemic sclerosis. Due to the rarity of NS, it is important to recognize these lesions, if presenting early in disease progression, as a sign of underlying scleroderma before internal organ damage becomes apparent. 

Case Presentation
A 42-year-old female with a history of systemic sclerosis presented with an enlarging left supraclavicular nodule. Upon physical exam, a 1.1cm erythematous nodule was found. The patient had clinical findings consistent with systemic sclerosis, including Raynaud’s phenomenon, gastroesophageal reflux disease, sclerodactyly, dyspnea on exertion, and skin tightening. The patient had been treated with CellCept 1500mg twice daily and responded very well to therapy. Pertinent laboratory data included speckled antinuclear antibodies (1:320 titer) and positive anti-RNA polymerase III antibodies (>80 units). 

The lesion was initially excised to rule out diagnosis of a cyst. However, histopathological analysis demonstrated a nodular proliferation of dense fibrotic collagen with scattered bland fibroblasts. A sparse perivascular and periadnexal lymphocytic infiltrate in the dermis was present with notable eccrine coil trapping. A CD34 stain showed markedly diminished dermal dendritic cells within the fibrotic nodule. Considering the patient’s known history of anti-RNA polymerase III-positive systemic sclerosis and histopathological findings, the focal lesion was consistent with nodular scleroderma. No subsequent treatments were performed.

Discussion
Nodular scleroderma is an atypical cutaneous presentation of systemic sclerosis and patients who are well-controlled on immunosuppressive therapy with few to no signs of internal organ disease are still susceptible to developing these lesions. Thus, diagnosis and treatment of NS remains a challenge. NS usually presents as 2 mm - 3 cm firm nodules or plaques resembling keloid or morphea, usually distributed on the trunk, neck, proximal extremities, and face. The pathogenesis of NS is also not well understood. Previous reports have shown that there may be a role for increased expression of connective tissue growth factor, tenascin, epidermal growth factor, or transforming growth factor beta in the formation of NS. 

Furthermore, treatment of NS is difficult as these lesions are resistant to many non-surgical interventions and remain stable over time. As in our case, surgical excision of NS has been the only treatment reported so far that results in full removal of NS, but not necessarily prevention of NS recurrence.


Systemic Sclerosis with a Rare Presentation of Nodular Scleroderma

Lymphomatoid papulosis (LyP) is a chronic cutaneous CD30-positive lymphoproliferative disorder that is characterized by red-to-violaceous crops of papulonodular skin lesions. Although LyP carries a positive prognosis, there is consensus that LyP is associated with a history of or subsequent development of lymphoma. Herein, we present a case of LyP in a patient with a history of cutaneous B-cell lymphoma. The patient is a 67-year-old male with a history of treated cutaneous B-cell lymphoma two years prior to presentation, currently in remission, presenting with a two week history of painful hemorrhagic papules on his hands. Biopsy revealed a dense lymphomatoid infiltrate with admixed histiocytes and dermal hemorrhage. Tumor cells expressed CD3 and CD4, with marked loss of CD7. Small clusters of CD30+ cells were noted. There was no significant expression of CD20, CD56, or myeloperoxidase. At subsequent follow up, his original lesions were healing, but he had developed a new crop of lesions on the foot and both hands. Given the patient’s clinical course and histopathologic findings, a diagnosis of LyP subtype C was favored. The patient’s presentation was notable due to antecedent history of cutaneous B cell lymphoma, in addition to confinement to acral sites.

Lymphomatoid Papulosis Type C in Patient with History of Cutaneous B-Cell Lymphoma

Background
Drug-induced hypersensitivity syndrome (DIHS) is a severe, potentially life-threatening drug reaction with cutaneous and internal organ involvement. DIHS can lead to various autoimmune conditions, occurring in about 10% of patients, including thyroiditis. This case highlights the need for post-DIHS thyroiditis screening.

Case Presentation
A 32-year-old woman presented to the emergency department with fever, morbilliform eruption, facial swelling, lymphadenopathy, and laboratory studies notable for atypical lymphocytes (4%), eosinophilia (1400), and aminotransferase abnormalities (AST 61, ALT 265). Two weeks prior, she initiated a course of oral trimethoprim-sulfamethoxazole. History, clinical exam, and laboratory findings were consistent with DIHS. She was treated with high-dose corticosteroids with a resolution of the eruption and stabilization of hepatic enzymes. At follow-up in an outpatient dermatology clinic four weeks after discharge, she was noted to have clinically painful thyromegaly, fever, tachycardia, and palpitations. She was referred to the ED to expedite workup for suspected symptomatic hyperthyroidism. Workup revealed TSH of 0.01 (L), FT4 >7.7 (H), TSI <0.10, TRAb negative, and TPO Ab 202 (H). Thyroid ultrasound showed enlarged thyroid with heterogenous echotexture. Endocrinology diagnosed her with thyroiditis and prescribed atenolol for symptom management. She continued to have heat intolerance, palpitations, and faint pink macules on her lower legs. She initiated low-dose methimazole with a resolution of symptoms.

Discussion

One of the most common drug classes to cause post-DIHS autoimmune thyroiditis are sulfa drugs and antiepileptics. The most commonly seen issues that arise in the thyroid post-DIHS are Hashimoto’s thyroiditis and Graves’ disease, which have been observed to occur between 2 months and 3 years after the diagnosis of DIHS. As seen in this case, the patient experienced debilitating symptoms of thyroiditis within 1 month, which could have been minimized with early screening and recognition. This case emphasizes the importance of dermatologic follow-up for patients diagnosed with DIHS and the role of post-DIHS screening for autoimmune thyroid disease, as missed diagnosis of delayed sequelae can lead to significant morbidity.

Importance of Post-Drug Induced Hypersensitivity Syndrome Thyroiditis Screening

Aching Adipose: Shedding Light on Dercum’s Disease

Lip and face lesions are recognized in dermatology as significantly distressing for patients. There are stereotypes associated with different lesions in this area that affect patients deeply, motivating them to seek care immediately. The average population or clinician is aware of common lip lesions, the most diagnosed of which is herpes simplex virus. Dermatologists must continue to be aware of unique lip and face lesions as they may grow rapidly, be worrisome, and affect the patient’s perception of themselves and psychological well-being. We present the case of neutrophilic sebaceous adenitis, which is an incredibly rare condition that has been reported minimally in the literature. Recognition of rapidly growing lesions, like our patients, is vital for proper diagnosis and treatment.
A 74-year-old female presented to the dermatology clinic with a 5-day history of a rapidly progressive lower lip lesion. Valacyclovir and Carmex only minimally improved her condition. Examination was significant for tenderness to palpation. The patient had no other lesions, no preceding trauma or bites, and no systemic symptoms. Histopathology showed a somewhat dense, superficial and deep, perivascular and periadnexal infiltrate composed predominantly of lymphocytes. The overlying epidermis showed some interface changes with a few necrotic keratinocytes as well as a focal but prominent neutrophilic infiltrate involving a sebaceous gland lobule. No increased dermal mucin was identified on Alcian blue stain. The patient discontinued valacyclovir and treated the lesion with triamcinolone 0.1% twice a day for a few weeks until it cleared. 
Neutrophilic sebaceous adenitis is a very rare condition. Pathogenesis is unknown but triggers such as sun exposure, antibiotics, and febrile illness have been reported. Lesions can progress over days to months and are typically asymptomatic. Recognition of this lesion around the lips and face is imperative as a differential diagnosis for providers to be aware of. Histopathology typically shows an inflammatory infiltrate, particularly of neutrophils and lymphocytes, around sebaceous glands. Sebocytes may be necrosed and further inflammatory infiltrates may be noted around the dermis. There are no current dermatologic guidelines on management although case reports have shown successful treatment with topical or oral steroids, metronidazole, or isotretinoin.

Looking Beyond the Ordinary Lip Lesion: A Rapidly Progressive Case of Neutrophilic Sebaceous Adenitis

Dermatofibromas are benign cutaneous soft-tissue tumors characterized by solitary or multiple, firm red-brown to black dermal nodules found on the trunk or extremities. Dermatofibromas most frequently affect middle-aged adults and have a slight preponderance in females. Variants of dermatofibroma include hemosiderotic, aneurysmal, epithelioid and deep fibrous histiocytomas, cellular, lipidized, lichenoid, keloidal, atrophic, and atypical. 

Diagnosis of the classical type of dermatofibroma is made clinically and based on dermoscopic patterns; however, the clinical and histologic heterogeneity observed in dermatofibroma variants can pose a diagnostic challenge and may be difficult to distinguish from potentially aggressive malignancies. Notably, hemosiderotic dermatofibroma has been reported to clinically simulate melanoma, while atypical dermatofibroma, is often mistaken histologically for sarcoma. 

Herein, we report a unique presentation of a patient with a recurrent atypical melanocytic nevus and multiple benign dermatofibromas masquerading as potentially malignant counterparts.

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A Case of Dual Left Anterior Descending Coronary Artery Supply with a Myocardial Bridge

Next from 2023 AMA Research Challenge

An Exceedingly Rare Case of Deglutition-Induced Atrial Fibrillation