The Management of Erythrodermic Psoriasis Complicated by Cyclosporine.

We present a 64-year-old woman with past medical history of psoriasis and alcoholic liver cirrhosis who presented with a diffuse, erythematous, and scaly rash. Pertinent medications included topical triamcinolone 0.1% cream. She was started on oral prednisone 40 milligrams (mg) and oral cyclosporine 150 mg daily and was continued on topical triamcinolone. After the administration of two doses of this regimen, the serum creatinine increased to 1.76 mg/dL, and serum potassium increased to 6.7 mEq/L. The serum creatinine continued to uptrend to 2.42 mg/dL, and the glomerular filtration rate (GFR) decreased to 20 mL/min. The patient was emergently hemodialyzed. The patient was placed on an extended steroid taper, alleviating the psoriatic rash. However, the patient needed to be placed on a steroid-sparing regimen. Because of its rarity and ensuing complications, erythrodermic psoriasis must be identified and managed promptly. Cyclosporine is currently the first-line treatment. However, initiation of this therapy in our patient resulted in an acute kidney injury (AKI). Even though a steroid taper assisted in alleviating erythroderma, a steroid-sparing regimen needed to be started. This led to the consideration of alternate methods of therapy for further management of erythrodermic psoriasis with renal impairment.

Reactive Perforating Collagenosis; An Uncontrolled Pruritus That Left You Scratching Your Head.

Acquired perforating collagenosis is a rare disease of altered collagen formation that is extruded through the epidermis. It is most commonly seen in patients with microvascular disease including longstanding diabetes and chronic kidney disease (CKD). Due to the rarity of the disease, no large randomized clinical studies have been performed to determine the most efficacious method of treatment. Therefore, most of the knowledge available for treatment is secondary to the information collected through case reports, case series, and retrospective analyses. In this report, we present the case of a 68-year-old male with history of stage IV CKD who presented with a severe skin rash that was present over his body, including the chest, arms, back, neck, and buttocks. It did not involve the mouth, legs, palms or soles of the feet. He did not have a significant history of diabetes and had been placed on steroids with the concern that this rash may have been secondary to a drug reaction, erythema multiforme, or bullous pemphigoid. Two skin biopsies were performed as the patient was not responding to systemic and topical steroid or oral antibiotic therapy. The final biopsy ultimately revealed a diagnosis of acquired perforating collagenosis. This is unusual in our case because although our patient had advanced CKD, he was not on dialysis, and had no significant longstanding history of diabetes. Additionally, as the prevalence of CKD is increasing in the population, it becomes more pertinent for providers to be aware of dermatological conditions associated with advanced CKD. This case report seeks to raise awareness of this disease. Furthermore, as the initial skin biopsy was unrevealing, this case also emphasizes the importance of repeating a biopsy to reduce the chance of sampling error.

Mucosal Melanoma: A Rare Entity and Review of the Literature.

Mucosal melanoma is a rare variant of melanoma representing around 1% of total cases of melanoma diagnosed. The usual sites of mucosal involvement are the sino-nasal passages, the oral cavity, and less commonly the upper gastrointestinal (GI) tract. It also has been reported to occur in vulvovaginal and anorectal mucosa.  We present a rare case of mucosal melanoma that presented as recurrent epistaxis, headache, and sinus pressure. CT maxillofacial imaging revealed a large mass right nasal cavity. This was biopsied by ENT and shown to be mucosal melanoma. This was treated with palliative radiation followed by immunotherapy with nivolumab.  Along with details of the case, we also discuss current treatment options with a focus on the role of immunotherapy and its efficacy in cases of head and neck mucosal melanoma. Our review of literature supports use of combination immunotherapy (including both nivolumab and ipilimumab) as it shows greater efficacy than either therapy alone. When combined with radiation therapy (RT) the overall response rate is improved and RT induces an abscopal effect; where benefits of RT are also seen at nonirradiated locations.  In our patient, the use of radiation was essentially palliative as the patient was deemed to not be a surgical candidate. We discuss in our literature review the optimum timing of radiation in relation to definitive surgery or immunotherapy.

Benign but Terminal: Cardiopulmonary Collapse from a Massive Chest Wall Lipoma.

Lipomas are the most common benign soft tissue tumor. Yet, strikingly simple tumors can become problematic when compounded by odd characteristics such as size and location. We report the case of a 53-year-old male who developed complete right lung collapse secondary to a large right-sided chest wall lipoma with accelerated growth in the past 6 months. Bronchoscopy revealed extrinsic compression of the right mainstem bronchus. Histopathology of the soft tissue mass was suggestive of a lipoma. The mass was not amenable to surgery due to a high risk of mortality from his underlying comorbidities. His hospital stay was complicated by progressive end-stage restrictive lung disease necessitating intubation and eventually a tracheostomy, recurrent pneumonias, multiorgan dysfunction, and his eventual demise. We highlight a rare presentation of an unchecked lipoma, which ultimately led to the death of our patient. Simple lipomas show insidious growth and can remain asymptomatic until they reach a large size. Chest wall tumors should be considered malignant until proven otherwise by excisional biopsy. This reiterates the need to treat all chest wall tumors with wide resection in order to provide the best chance for cure.

Varicella-Zoster Meningitis With Hypoglycorrhachia in an Immunocompetent Patient Presenting With Disseminated Varicella-Zoster Infection.

Varicella-zoster virus (VZV) infection is rarely reported in immunocompetent hosts. We report the case of a 40-year-old male who presented with altered mental status. One week prior, he was seen at his outpatient physician's office for a rash along the lateral right thigh. Erythema of the right gluteal region was noted, but no vesicles were present. He was treated for shingles rash with acyclovir with improvement. After a period of initial improvement in the rash, the patient developed a persistent headache. Given his migraine history, he overlooked the headache. He then developed fever, followed by confusion and was brought to the ED for further evaluation. CT head was unremarkable. Lumbar puncture revealed aseptic meningitis. This case highlights the unusual presentation of disseminated VZV infection in an immunocompetent host. It stresses the importance of maintaining high suspicion for disseminated VZV infection despite the patient being immunocompetent.

Primary Sinonasal Lymphoma: A Rare Cause of Cranial Neuropathies.

Primary sinonasal lymphomas are a rare type of non-Hodgkin lymphoma (NHL) with an overall incidence of about 1% of all head and neck cancers. Diffuse large B-cell lymphoma (DLBCL) is the most common type of NHL and it most commonly occurs in elderly men. The diagnosis of such a lymphoma is difficult because of its varied presentation, which usually occurs late after a significant mass effect has taken place. Symptoms vary significantly, from simply nasal obstruction or epistaxis to varied cranial neuropathies or the confusion seen with central nervous system (CNS) spread. Patients may present with a large orbital mass and proptosis. Therefore, emphasis should be placed on earlier detection by using appropriate imaging modalities to reveal such masses. A biopsy is necessary to confirm the diagnosis. The prognosis is scored by the International Prognostic Index. Staging scans with whole-body computed tomography (CT) with contrast and positron emission tomography-fluorodeoxyglucose (PET-FDG) are required to determine other areas of involvement. Treatment is with R-CHOP (rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone) with the case-dependent use of intrathecal chemoprophylaxis (methotrexate) to prevent CNS spread. Here, we present a case series of two patients who were found to have a primary sinonasal lymphoma of the DLBCL type. In both cases, the presenting symptoms were vague. A high index of suspicion is required to diagnose NHL early on, which portends the best chance of a successful outcome. This article seeks to emphasize the role of including primary sinonasal lymphoma as a differential in the presentation of unrelenting cranial neuropathies or facial mass.

Anti-NMDAR-Positive Small-Cell Lung Cancer Paraneoplastic Limbic Encephalitis: A Case Report and Literature Review.

INTRODUCTION: Paraneoplastic limbic encephalitis (PLE) is a rare disease that presents as rapid onset dementia characterized by short-term memory loss (STM), anxiety, and behavioral changes. Anti-NMDAR antibodies are unfrequently reported in PLE associated with small-cell lung cancer (SCLC). Given that PLE can precede the diagnosis of cancer, it is very important that once infectious, metabolic, nutritional, or structural disorders associated with short-term memory loss are ruled out that vigorous effort must be made to rule out underlying malignancy. CASE: We report a rare case of PLE as the presenting symptom of SCLC. A 72-year-old male with history of COPD was brought to the ED by his wife after he was found to have short-term memory loss, including forgetfulness of his wedding anniversary the day before, and anxiety. Neurological exam showed impaired short-term recall on MOCA. CT head showed no evidence of infarct. Lumbar puncture was performed which showed lymphocytic pleocytosis, a nonspecific inflammatory change. CSF panel was negative for HSV, Neisseria, Hemophilus, E. coli, and HIV. Initial EEG was unremarkable, though a repeat EEG showed mild slowing of the posterior dominant rhythm consistent with mild encephalopathy. MRI showed equivocal increased FLAIR on T2-weighted images in the bilateral temporal lobes, left greater than right. CTA thorax showed bulky mediastinal and right hilar LAD. FNA of the R4 lymph node revealed SCLC. The NM bone scan showed no osteoblastic lesions. While the serum autoantibody panel was positive for anti-NMDAR, the CSF autoantibody panel returned entirely negative. Chemotherapy with etoposide and cisplatin was started on Day 4 of admission. The patient's neurological symptoms showed improvement following chemotherapy. CONCLUSION: This case highlights the importance of recognizing short-term memory loss as a feature of PLE.

A Rare Cause of Bilateral Corneal Ulcers: Vitamin A Deficiency in the Setting of Chronic Alcoholism.

Vitamin A deficiency is rarely encountered in the western world. When encountered, vitamin A deficiency is seen as a component of the malabsorption spectrum of disease. Given the infrequency of nutritional deficits in the developed world, vitamin A-associated ophthalmologic disease is rarely encountered. We report a case of a 56-year-old male with severe vitamin A deficiency in the setting of alcoholic liver cirrhosis. This case emphasizes two important points. First, it considers vitamin A deficiency as a cause of corneal ulceration in patients with chronic alcoholism. Second, it raises awareness of hepatotoxicity that can result after the supplementation of vitamin A in patients with chronic alcoholism. Although an uncommon diagnosis, it should be considered when other causes, such as infectious and autoimmune conditions, are ruled out.

Salty, Sweet and Difficult to Treat: A Case of Profound Hypernatremia in the Setting of Hyperosmotic Hyperglycemic State.

Hyperosmolar hyperglycemic state (HHS) is a disorder that occurs most frequently in type 2 diabetics and is associated with high mortality - up to 50%. Hypernatremia, when associated with HHS, worsens the prognosis. Encephalopathy is evident at a serum sodium level greater than 160 mOsm/kg. Additional symptoms include lethargy, weakness, seizures, and coma. Rhabdomyolysis can rarely occur in hyperosmolar states. Here we describe a case of severe hypernatremia in the setting of HHS leading to profound encephalopathy and report to the best of our knowledge the highest serum sodium level published in the literature. A 50-year-old female with no past medical history (PMH) of diabetes presented to the ED obtunded and found to have a glucose level of 1400 mg/dL without metabolic acidosis or ketosis. Her sodium on presentation was 169 mOsm/kg but subsequently rose to 200 mOsm/kg when corrected for hyperglycemia. Plasma osmolality was 340 mOsm/kg. She developed pre-renal acute kidney injury (AKI) secondary to the osmotic diuresis from severe hyperglycemia as well as rhabdomyolysis with a peak creatine kinase(CK) level of 2493. The free water deficit was 14L which was corrected. New-onset anisocoria raised concern for osmotic demyelination which was further investigated with MRI. An acute ischemic stroke in the right caudate was found. Fortunately, the patient survived the endocrine emergency. This case emphasizes the importance of an appropriate rate of sodium correction. This case is particularly unique because the degree of hypernatremia seen here was in the absence of intentional salt loading (for example by the administration of hypertonic saline), or psychiatric disease (as psychogenic adipsia). In conclusion, we report the case of severe hypernatremia and the highest documented serum sodium level was seen in literature in the background of HHS, rhabdomyolysis and septic shock.

Sodium-Thiosulfate Induced Life-Threatening Metabolic Acidosis Limiting Treatment of Calciphylaxis.

BACKGROUND Calcific uremic arteriolopathy (CUA) is a rare and incredibly painful cutaneous disorder secondary to microvascular involvement in which calcium dysregulation leads to stenosis of medium sized arterial blood vessels along with endothelial dysregulation and thrombosis. Ultimately, these patients are at high risk for non-healing wounds with risk of death from sepsis and multi-organ failure. It is a poorly understood condition with limited therapies that do not offer mortality benefit. Prevalence is about 4% in hemodialysis patients. Sodium thiosulfate (STS) can be used in hemodialysis patients but therapy is often limited by the development of high anion gap metabolic acidosis. CASE REPORT A 53-year-old male who had end stage renal disease and who was on hemodialysis and taking warfarin for bio-prosthetic mitral valve replacement and atrial fibrillation presented with non-healing right lower extremity cellulitis which had failed outpatient treatment. A skin biopsy of the lesion was consistent with CUA. The patient failed to improve on calcitriol and cinacalcet and was started on intravenous STS. Subsequently, he developed life threatening metabolic acidosis requiring a bicarbonate drip. He died 12 weeks after his initial diagnosis of CUA. CONCLUSIONS This article seeks to describe how the treatment of CUA; a rare disease with high mortality, is limited by the development of metabolic acidosis when using STS therapy. There is an 80% mortality rate within 6 months from CUA with major adverse effect of a high anion gap metabolic acidosis. Further research is needed in the field of establishing optimal dosing and frequency.

Primitive neuroectodermal tumor of the pancreas.

Primitive neuroectodermal tumors (PNETs) are rare small round cell malignancies closely related to Ewing's sarcoma. Involvement of the abdominal cavity, specifically the pancreas, is extremely rare. PNETs affect predominantly children and young adults. The clinical presentation is mostly vague, with a short history of symptoms even in metastatic disease. Findings on imaging studies are nonspecific. The diagnosis can be suggested by the microscopic appearance of the tumor cells, but should be confirmed by histology, immunohistochemistry, fluorescence in situ hybridization, immunoreactivity evaluation of MIC2-protein (CD99) expression, and when possible testing for the chromosome translocation t(11;22) (q24,q12). In adults, the prognosis is poor with no standard treatment. Here, we present a case of pancreatic PNET in a 61-year-old man who presented with persistent abdominal pain and weight loss.

Nivolumab and Ipilimumab-induced Acute Inflammatory Demyelinating Polyradiculoneuropathy: A Case Report.

Immunotherapies such as the cytotoxic T-lymphocyte-associated protein 4 inhibitor ipilimumab and the programmed cell death protein 1 inhibitor nivolumab have become ubiquitous in cancer treatment. Recently, the FDA approved nivolumab with or without ipilimumab for the treatment of refractory small cell lung cancer. Immunotherapies increase the immune response to cancer cells by interfering with inhibitory molecular pathways that prevent tumor cell killing, thus augmenting tumor cell death without many of the cytotoxic side effects associated with chemotherapy. However, this augmented immune response may result in unwanted immune-mediated inflammation of different organs and are therefore associated with immune-related adverse events, unlike traditional chemotherapies or targeted therapies. Here, we describe 1 patient with advanced small cell lung cancer who developed grade III-IV acute inflammatory demyelinating polyradiculoneuropathy after treatment with ipilimumab and nivolumab. The patient was treated with intravenous immunoglobulin alone and showed symptomatic improvement.

Chilaiditi's Sign: A Case Report.

Chilaiditi's sign refers to the interposition of the colon (usually the transverse colon) between the diaphragm and the liver. When associated with abdominal pain it is referred to as Chilaiditi's syndrome. Chilaiditi's sign is rare entity with an estimated incidence of 0.025 to 0.28% worldwide. The sign occurs more frequently in males, with a male to female ratio of 4:1. Apparent pneumoperitoneum seen on imaging below the right hemidiaphragm, a life-threatening condition, may in fact be merely Chilaiditi's sign. Awareness of this phenomenon and its consideration as a differential diagnosis is essential to prevent unnecessary laparoscopic intervention. Here we present a case of a 74-year-old male who was incidentally found to have free air under the diaphragm without symptoms of abdominal pain. After further evaluation by the radiologists and surgeons it was concluded that he had Chilaiditi's sign and no further intervention was required. However, due to the lack of awareness of this radiographic finding patients can be subjected to unnecessary surgical intervention.

Strengthening Primary Care Through Family Medicine Around the World Collaborating Toward Promising Practices.

BACKGROUND AND OBJECTIVES: There is a limited evidentiary base on the development of family medicine in different contexts and countries. The lack of evidence impedes our ability to compare and characterize family medicine models and identify areas of success that have led to the effective provision of care. This paper offers a comparative compilation and analysis of the development of family medicine training programs in seven countries: Brazil, Canada, Ethiopia, Haiti, Indonesia, Kenya, and Mali. METHODS: Using qualitative case studies, this paper examines the process of developing family medicine programs, including enabling strategies and barriers, and shared lessons. An appreciative inquiry framework and complex adaptive systems thinking inform our qualitative study. RESULTS: Committed partnerships, the contribution of champions, health policy, and adaptability were identified as key enablers in all seven case studies. The case studies further reveal that some enablers were more salient in certain contexts as compared to others, and that it is the interaction of enablers that is crucial for understanding how and why initiatives succeeded. The barriers that emerged across the seven case studies include: (1) resistance from other medical specialties, (2) lack of resources and capabilities, (3) difficulty in sustaining support of champions, and (4) challenges in brokering effective partnerships. CONCLUSIONS: A key insight from this study is that the implementation of family medicine is nonlinear, dynamic, and complex. The findings of this comparative analysis offer insights and strategies that can inform the design and development of family medicine programs elsewhere.

Assessing health program performance in low- and middle-income countries: building a feasible, credible, and comprehensive framework.

BACKGROUND: Many health service delivery models are adapting health services to meet rising demand and evolving health burdens in low- and middle-income countries. While innovative private sector models provide potential benefits to health care delivery, the evidence base on the characteristics and impact of such approaches is limited. We have developed a performance measurement framework that provides credible (relevant aspects of performance), feasible (available data), and comparable (across different organizations) metrics that can be obtained for private health services organizations that operate in resource-constrained settings. METHODS: We synthesized existing frameworks to define credible measures. We then examined a purposive sample of 80 health organizations from the Center for Health Market Innovations (CHMI) database (healthmarketinnovations.org) to identify what the organizations reported about their programs (to determine feasibility of measurement) and what elements could be compared across the sample. RESULTS: The resulting measurement framework includes fourteen subgroups within three categories of health status, health access, and operations/delivery. CONCLUSIONS: The emphasis on credible, feasible, and comparable measures in the framework can assist funders, program managers, and researchers to support, manage, and evaluate the most promising strategies to improve access to effective health services. Although some of the criteria that the literature views as important - particularly population coverage, pro-poor targeting, and health outcomes - are less frequently reported, the overall comparison provides useful insights.

Trans-national scale-up of services in global health.

BACKGROUND: Scaling up innovative healthcare programs offers a means to improve access, quality, and health equity across multiple health areas. Despite large numbers of promising projects, little is known about successful efforts to scale up. This study examines trans-national scale, whereby a program operates in two or more countries. Trans-national scale is a distinct measure that reflects opportunities to replicate healthcare programs in multiple countries, thereby providing services to broader populations. METHODS: Based on the Center for Health Market Innovations (CHMI) database of nearly 1200 health programs, the study contrasts 116 programs that have achieved trans-national scale with 1,068 single-country programs. Data was collected on the programs' health focus, service activity, legal status, and funding sources, as well as the programs' locations (rural v. urban emphasis), and founding year; differences are reported with statistical significance. FINDINGS: This analysis examines 116 programs that have achieved trans-national scale (TNS) across multiple disease areas and activity types. Compared to 1,068 single-country programs, we find that trans-nationally scaled programs are more donor-reliant; more likely to focus on targeted health needs such as HIV/AIDS, TB, malaria, or family planning rather than provide more comprehensive general care; and more likely to engage in activities that support healthcare services rather than provide direct clinical care. CONCLUSION: This work, based on a large data set of health programs, reports on trans-national scale with comparison to single-country programs. The work is a step towards understanding when programs are able to replicate their services as they attempt to expand health services for the poor across countries and health areas. A subset of these programs should be the subject of case studies to understand factors that affect the scaling process, particularly seeking to identify mechanisms that lead to improved health outcomes.