Clinical Case of the Month: A 57-Year-Old Man with an Axillary Mass.

A 57-year-old man presented to the surgical oncology clinic with a mildly tender mass under his right arm. Four years prior, the patient had a melanoma removed from his right shoulder along with an ipsilateral right axillary sentinel lymph sampling. Computed tomography (CT) scan was negative for metastatic disease at that time. The patient did not undergo completion axillary node dissection and was lost to follow-up. The patient was originally from Australia, did not tan but reported multiple sunburns before age 18. He was of Irish ancestry. He denied weight gain, fever, fatigue, anorexia, or night sweats. The patient had a medical history of atrial fibrillation, hypertension, gout, melanoma, and benign prostatic hypertrophy. His surgical history included an appendectomy and a facial laceration repair. His brother died at 16 years old from leukemia and his mother died from colon cancer. He consumed 3 alcoholic beverages per day and denied tobacco or illicit drug use. On physical exam, the patient's temperature was 98.8° Fahrenheit, heart rate of 73 beats / minute, blood pressure of 121 / 59 mm Hg, respiratory rate of 18 / min. He appeared to be healthy and in no apparent distress. Cardiovascular, respiratory, breast, gastrointestinal, musculoskeletal, and neurological exam were unremarkable. His right axillary lymph node exam revealed a firm mass roughly 2.5 cm tall by 1.5 cm wide. This mass was biopsied and findings were consistent with metastatic melanoma. CT scan revealed small volume mediastinal adenopathy and a 4.5 cm right axillary mass. There was a 4.7 cm lesion within the anterior left lower lobe of the liver and periportal node conglomerate measuring 3.9 cm consistent with metastatic disease (Figure 1). He was negative for the BRAF V600E mutation. The patient was consented for treatment with combination immune checkpoint inhibition with ipilimumab and nivolumab. After two cycles the patient showed good response, but temporarily stopped treatment after complications related to a ST segment elevation myocardial infarction. He developed mild pneumonitis felt to be related to nivolumab, and recovered after a short course of glucocorticosteroids. Restaging CT scans were ordered after two cycles of therapy (Figure 2), which showed decrease in the size of the axillary and hepatic metastases. At six months, CT scans showed continued durable response (Figure 3).

Approach to skin and soft tissue infections in non-HIV immunocompromised hosts.

PURPOSE OF REVIEW: Skin and soft tissue infections are frequent contributors to morbidity and mortality in the immunocompromised host. This article reviews the changing epidemiology and clinical manifestations of the most common cutaneous pathogens in non-HIV immunocompromised hosts, including patients with solid organ transplants, stem cell transplants, solid tumors, hematologic malignancies, and receiving chronic immunosuppressive therapy for inflammatory disorders. RECENT FINDINGS: Defects in the innate or adaptive immune response can predispose the immunocompromised host to certain cutaneous infections in a predictive fashion. Cutaneous lesions in patients with neutrophil defects are commonly due to bacteria, Candida, or invasive molds. Skin lesions in patients with cellular or humoral immunodeficiencies can be due to encapsulated bacteria, Nocardia, mycobacteria, endemic fungal infections, herpesviruses, or parasites. Skin lesions may reflect primary inoculation or, more commonly, disseminated infection. Tissue samples for microscopy, culture, and histopathology are critical to making an accurate diagnosis given the nonspecific and heterogeneous appearance of these skin lesions due to a blunted immune response. SUMMARY: As the population of non-HIV immunosuppressed hosts expands with advances in medical therapies, the frequency and variety of cutaneous diseases in these hosts will increase.

Clinical Case of the Month: PERSISTENT HYPERTENSION IN A YOUNG WOMAN: A CLASSIC PRESENTATION OF CONN'S SYNDROME.

Primary aldosteronism PA is a secondary cause of hypertension that is often missed due to inadequate clinical evaluation and the lack of classically described laboratory abnormalities. Based on guidelines from the Endocrine Society, primary aldosteronism should be suspected in young patients with moderate to severe hypertension, patients with hypertension and coexisting hypokalemia, any patient with hypertension and an incidental adrenal adenoma, and hypertension in the setting of a significant family history of early onset hypertension or cerebral vascular accident in a first degree relative less than 40 years of age.1 In previous years, primary aldosteronism was attributed to less than one percent of all causes of secondary hypertension. However, recent research and increased utilization of aldosterone plasma renin ratio ARR as a method for screening has led to the understanding that majority of patients with PA are not hypokalemic, and the current literature now places the incidence of PA between 5-13 percent. Additionally, a growing body of evidence has demonstrated inflammatory, fibrotic, and remodeling effects on the cardiovascular and renal tissue that appear to be independent of PA- induced hypertension. Therefore a high suspicion for PA must be incorporated into evaluation of hypertensive patients, as diagnosis and subsequent treatment not only improves blood pressure control, but also acts to diminish cardiovascular morbidity and mortality. Here we present a case of a young woman with a seven-year history of hypertension prior to receiving a diagnosis of Conn's Syndrome.

Clinical Case of the Month: Hypereosinophilia in a Young Woman with a History of Childhood Asthma.

Hypereosinophillia is a rare clinical entity. It is associated with a wide differential diagnosis including neoplasm, infection, and allergic etiologies. Clinicians should have a well defined approach to hypereosinophilia in order to find treatable causes. We present a case of hypereosinophillia caused by parasitic infection with Toxocara canis. We also review epidemiology, transmission, microbiology, and management of Toxocara canis.

Clinical Case of the Month: A 48-Year-Old Man With Fever and Abdominal Pain of One Day Duration.

A 48-year-old man residing in a mental health department inpatient program with a history of schizoaffective disorder presented to the emergency department with a chief complaint of fever and intense abdominal pain for one day. The patient stated he initially fell in the shower and afterwards experienced back pain. He was transferred to an acute care unit within the facility for further evaluation. The facility physician noted that the patient had a mild temperature elevation and abdominal rigidity on exam. At that time, he was given two doses of benztropine intramuscularly, and transferred to our hospital for further evaluation. The patient exhibited fever, diffuse abdominal pain and a nonproductive cough, but denied chills, dysuria, urinary frequency, hematuria, weakness, diarrhea, melena or hematochezia. He did have a one-week history of constipation for which he was given sodium phosphate enemas, magnesium citrate and docusate sodium, eventually resulting in a bowel movement. He also complained of new onset dysphagia. There were no recent changes to his medications, which included clonazepam, divalproex sodium extended release, olanzapine and risperidone. He denied use of tobacco, alcohol or illicit drugs.

Clinical Case of the Month: A 49-Year-Old Man with Fever, Headache and Leg Weakness.

A 49-year-old man with an unremarkable past medical history presented to an outside hospital with a five-day history of fever, left leg weakness, myalgia and headache. The patient reported that the illness started as a fever and sore throat and he was originally diagnosed with streptococcal pharyngitis and prescribed antibiotics. The day after his initial diagnosis, his fever had progressed to include a headache, myalgia, a rash on his upper torso and right shoulder and sudden-onset left leg weakness with preserved sensation. With progressively worsening symptoms, he eventually presented to a local emergency department (ED), five days after his symptoms first started. He was experiencing continued left leg weakness, an inability to ambulate, persistent fevers to 103ºF, muscle aches, an intense band-like headache and confusion. The patient denied neck stiffness, photophobia, loss of sensation or any additional muscle weakness. He denied any recent travel aside from work, any sick contacts, recent tick/insect bites, history of sexually transmitted diseases or contact with animals. He reported no history of illicit drug use as well as no recent weight loss, trauma or radiation exposure. The patient had approximately a 10-pack-year tobacco smoking history. For the last ten years he drank about a six-pack of beer daily while onshore (roughly two weeks out of every month). He works on an offshore oil platform. He was not taking any home medications besides his recently prescribed antibiotics. He lived alone at home in a moderately rural area of South Louisiana. His family history was non-contributory.

Clinical case of the month: a 22-year-old man with AIDS presenting with shortness of breath and an oral lesion.

Since the development of combination antiretroviral therapy (cART), the incidence and mortality associated with Kaposi sarcoma (KS) have been reduced, although not eliminated. Clinical presentations of KS range from simple skin involvement to disseminated disease, including involvement of the oral cavity and viscera, which portends a more ominous prognosis. Multiple case reports and data from clinical trials indicate that administration of systemic corticosteroids may aggravate KS. We present a case of disseminated KS following administration of prednisone for presumed immune reconstitution inflammatory syndrome (IRIS) associated with fungal pneumonia in an HIV-infected individual. The discussion that follows outlines the pathophysiology and clinical presentations associated with KS and existing data for the role of corticosteroids in promoting KS progression.

Clinical Case of the Month. A 44-Year-Old HIV-Infected Man With Righ-Shoulder Swelling.

Immunocompromised patients are susceptible to various joint infections with less-common pathogens, such as mycobacterium. Physicians should have a low threshold to investigate the cause of an arthropathy further. An aspiration of the effusion is usually warranted to identify the possible pathogen and target treatment. We report an unusual presentation of a human immunodeficiency virus-infected patient with a chronic effusion arthropathy of his right shoulder due to Mycobacterium kansasii. We review the risk factors, transmission, clinical manifestations, and management of Mycobacterium kansasii.

CLINICAL CASE OF THE MONTH. A 63-Year-Old Woman With Rash and Proximal Muscle Weakness.

A 63-year-old woman with a history of infiltrating ductal breast cancer, status post-mastectomy and chemotherapy, was in remission for 18 months prior to being admitted to the hospital with complaints of a pruritic erythematous macular rash involving her head, chest, and bilateral upper and lower extremities. Along with the dermatologic manifestations, physical exam revealed proximal symmetrical muscle weakness and bilateral axillary lymphadenopathy. Initial workup for muscle weakness revealed a creatine kinase of 2,200 IU/L (normal 20-180 IU/L). After administration of intravenous fluids for renal protection, serum sodium dropped to 121 mEQ/L (normal 135-145 mEQ/L). Computed tomography of the chest showed axillary and supraclavicular lymphadenopathy. Biopsy of a supraclavicular node revealed infiltrating ductal cancer with histologic and morphologic characteristics similar to her previous breast cancer. Following an extensive laboratory workup, we concluded that our patient's myositis and hyponatremia were paraneoplastic syndromes secondary to her recurrent breast cancer.

A case of disseminated cryptococcosis with skin manifestations in a patient with newly diagnosed HIV.

In this case report, we provide a concise review of the pathogenesis, clinical presentation, key diagnostic findings, and treatment options for Cryptococcus. Cryptococcus neoformans is a ubiquitous dimorphic fungus that can potentially be life threatening, especially in immunocompromised hosts. It commonly infects the respiratory tract or central nervous system. However, skin manifestations can be a marker of severe and disseminated disease, particularly in individuals at higher risk, including those patients with AIDS, cancer, long-term corticosteroid use, and organ transplant recipients. A timely diagnostic workup, including skin biopsy,blood cultures, and lumber puncture, is crucial since skin lesions may mimic other infectious processes. In addition, prompt, appropriate antifungal treatment should be initiatedto decrease morbidity and mortality.

Clinical case of the month. A 50-year-old man with a persistent rash.

In September 2006, the United States Centers for Disease Control and Prevention (CDC) published updated recommendations for routine opt-out human immunodeficiency virus (HIV) testing in all healthcare settings. As late diagnosis of infection increases individual and societal risks, a goal of the 2010 National HIV/AIDS Strategy is to increase the percentage of those aware of their infection. In 2008, two years following changes in the CDC testing recommendations, 44.6% of individuals 18-64 years of age reported a history of having a HIV test, resulting in close to 40,000 new HIV diagnoses. Emergency departments accommodate more than 120 million patient visits per year in the United States and can be the only point of contact individuals have with the healthcare system, particularly in uninsured and marginalized groups. Further implementation of opt-out testing can result in earlier diagnosis and improved health outcomes at the both the individual and public health levels.

Normocalcemic primary hyperparathyroidism-characteristics and clinical significance of an emerging entity.

The epidemiology and presentation of primary hyperparathyroidism (PHPT) is changing. Full-blown disease in its tertiary stage is rarely seen. Instead, asymptomatic hypercalcemia draws more attention because of the widespread use of screening laboratory tests. In recent years, clinicians have started ordering intact parathyroid hormone (iPTH) assays for a variety of reasons (eg, during the evaluation of fractures, nephrolithiasis, osteomalacia and low bone mass), even in individuals who have no evidence of hypercalcemia. The increased levels of iPTH without an underlying etiology may represent an early phase, or a distinct subtype, of PHPT, termed as normocalcemic primary hyperparathyroidism (NPH). The prevalence and clinical significance of NPH are unknown and are a matter of great debate. No guidelines regarding management of this entity exist. The authors describe a patient who had increased levels of iPTH, osteopenia and normal calcium and vitamin D. There was no discernible secondary cause for hyperparathyroidism (renal insufficiency, osteomalacia, vitamin D deficiency, lithium use, etc), and no intervention was offered aside from periodic monitoring of laboratory values and bone density. Analysis of data in patients with NPH should include elucidation of its natural history, temporal patterns in calcium level and prevalence of low bone mass, nephrolithiaisis and fragility fractures and could assist in devising evidence-based guidelines for management of the condition. Further characterization of NPH will be of benefit in defining its clinical impact and associated complications, to clarify the rationale behind the use of the iPTH assay as a screening test, and to assist in management of this entity.

Clostridium difficile infection in non-HIV-immunocompromised patients and in HIV-infected patients.

Clostridium difficile infection is a common cause of morbidity in patients with HIV infection and in patients with non-HIV immune deficiency. The frequency of C. difficile--associated diarrhea (CDAD) seen in these two patient populations has been attributed to immune deficiency, as well as to increased exposure to hospital environments and antibiotic therapy, both of which are major risk factors for CDAD. This article reviews recent data useful in the evaluation, treatment, and prevention of C. difficile infection in these patient groups. Recent publications relating to C. difficile infection in specific immunocompromised conditions are also discussed.

Clinical case of the month. A rare case of Budd Chiari syndrome.

Budd Chiari syndrome is a rare disorder resulting from hepatic venous outflow tract obstruction anywhere from the small hepatic veins to the suprahepatic inferior vena cava. This patient has a hypercoagulable state secondary to heterozygous mutation of factor V and the JAK2 mutation and is being anticoagulated. We hypothesize that the low protein C and low antithrombin III levels seen in this patient resulted from decreased synthetic function of the liver and were not indicative of actual deficiencies. Indeed, reports of coexisting protein C and antithrombin III deficiencies are not existent in the literature and likely are not compatible with life. All patients with BCS warrant a hypercoagulable work up and JAK2 mutation is increasingly recognized as a contributing factor, even in those patients without obvious signs of polycythemia vera.

Clinical case of the month. A 29-year-old man with acute onset blurry vision, weakness, and gait abnormality. Stroke.

A 29-year-old man, with no significant past medical history, was in his usual state of health until the afternoon of admission. The patient was seated at work eating lunch when he suddenly noticed that his vision became blurry. He covered his right eye and had no visual difficulty but noted blurry vision upon covering his left eye. At this point, the patient tried to stand up, but had difficulty walking and noticed he was "falling toward his left." Facial asymmetry when smiling was also appreciated. The patient denied any alteration in mental status, confusion, antecedent or current headaches, aura, chest pains, or shortness of breath. He was not taking any prescribed medications and had no known allergies. The patient denied any prior hospitalization or surgery. He denied use of tobacco, alcohol, or illicit drugs, and worked as a maintenance worker in a hotel. His family history is remarkable for his father who died of pancreatic cancer in his 50s and his mother who died of an unknown heart condition in her late 40s. Vital signs on presentation to the emergency department included temperature of 97.6 degrees F; respiratory rate of 18 per minute; pulse of 68 per minute; blood pressure of 124/84 mmHg; pulse oximetry of 99% on ambient air. His body mass index was 24 and he was complaining of no pain. The patient had no carotid bruits and no significant jugular venous distention. Cardiovascular exam revealed a regular rate and rhythm with no murmurs. Neurological exam revealed left-sided facial weakness, dysarthria, and preserved visual fields. He was able to furrow his brow. Gait deviation to the left was present, and Romberg sign was negative. Deep tendon reflexes were 2+ throughout, and no other focal neurological deficit was present. The patient was admitted to the hospital with a diagnosis of stroke. Electrocardiogram, fasting lipid profile, computed tomography (CT) scan of head, magnetic resonance imaging (MRI) of head and neck, and transthoracic echo with bubble study were ordered. The initial head CT did not reveal bleeding. He was started on aspirin (ASA). On the second hospital day, the symptoms improved with resolution of dysarthria. His ataxia had also improved. Fasting lipid profile revealed mildly elevated low-density lipoprotein and total cholesterol. His head MRI revealed an acute right thalamic stroke. Echocardiography was significant only for a patent foramen ovale (PFO) with transit of agitated saline "bubbles" from right atrium to left heart within three cardiac cycles (Figure). Doppler ultrasound of extremities revealed no evidence of deep venous thrombosis. A complete resolution of symptoms occurred by the third hospital day. The patient was discharged on full dose aspirin and a statin and was referred for consideration of enrollment in a PFO closure versus medical management trial.

Mycobacterium avium complex pulmonary disease: management options in HIV-negative patients.

OBJECTIVE: We present a case series and review of the literature of the management options in non-HIV-infected patients with Mycobacterium avium complex pulmonary disease (MAC-PD) with a focus on treatment failure and drug resistant disease. CASE SERIES: Five case histories are presented, depicting various clinical scenarios necessitating different approaches to therapy and highlighting the limitations and complications of these options. DISCUSSION: Mycobacterium avium complex (MAC) is well recognized as a significant cause of pulmonary disease in non-HIV infected patients and in those with intact immunity. Isolation of non-tuberculous mycobacteria (NTM) in culture is essential for the diagnosis of NTM lung disease. The typical presentation of MAC lung disease is apical fibrocavitary lung disease in men in their late 40s and early 50s who have a history of cigarette smoking and, frequently, excessive alcohol use. Other presentations of NTM lung disease include nodular bronchiectasis, solitary or multiple pulmonary nodules, and hypersensitivity pneumonitis. When indicated, the standard recommended treatment for most patients is a three-times-weekly regimen of clarithromycin or azithromycin, rifampin, and ethambutol with or without amikacin. Daily therapy is recommended for fibrocavitary disease. Based on published studies, macrolides are the only agents used for treatment of MAC disease for which there is a correlation between in vitro susceptibility and in vivo (clinical) response. Data regarding treatment of macrolide-resistant MAC (MRMAC) and multi-drug resistant MAC (MDRMAC) is sparse. Several drugs have been evaluated in drug-resistant MAC and have potential as effective therapy. Use of multiple drugs to which the isolate is susceptible is preferred to avoid development of future resistance. Surgery in mycobacterial disease is technically difficult, but selected patients with focal disease do benefit from resection of the involved lung. CONCLUSIONS: MAC has protean pulmonary manifestations, especially in those with no recognizable impairments in their immune system. Drug treatment, however, remains difficult with high failure rates and poor long-term sputum conversion. This case series is based on our clinical experience highlighting treatment options and the often unrecognized morbidity and mortality of severe, progressive MAC-PD. It underscores the need for increased awareness of MAC-PD and MDRMAC and the difficulties encountered in their management.