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.

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.

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.

Implementing malaria control in South Africa, Eswatini and southern Mozambique during the COVID-19 pandemic.

The COVID-19 pandemic has strained healthcare delivery systems in a number of southern African countries. Despite this, it is imperative that malaria control and elimination activities continue, especially to reduce as far as possible the number and rate of hospitalisations caused by malaria. The implementation of enhanced malaria control/elimination activities in the context of COVID-19 requires measures to protect healthcare workers and the communities they serve. The aim of this review is therefore to present innovative ideas for the timely implementation of malaria control without increasing the risk of COVID-19 to healthcare workers and communities. Specific recommendations for parasite and vector surveillance, diagnosis, case management, mosquito vector control and community outreach and sensitisation are given.

Maintaining focus on administering effective malaria treatment during the COVID-19 pandemic.

As September marks the start of the malaria season in South Africa (SA), it is essential that healthcare professionals consider both COVID- 19 and malaria when a patient who lives in or has recently travelled to a malaria area presents with acute febrile illness. Early diagnosis of malaria by either a rapid diagnostic test or microscopy enables prompt treatment with the effective antimalarial, artemether-lumefantrine, preventing progression to severe disease and death. Intravenous artesunate is the preferred treatment for severe malaria in both children and adults. Adding single low-dose primaquine to standard treatment is recommended in endemic areas to block onward transmission. Use of the highly effective artemisinin-based therapies should be limited to the treatment of confirmed malaria infections, as there is no clinical evidence that these antimalarials can prevent or treat COVID-19. Routine malaria case management services must be sustained, in spite of COVID-19, to treat malaria effectively and support SA's malaria elimination efforts.

Implementing malaria control in South Africa, Eswatini and southern Mozambique during the COVID-19 pandemic

The COVID-19 global pandemic reached South Africa (SA), Mozambique and Eswatini in March 2020.[1] Since then an exponential increase in SARS-CoV-2 infections has severely stretched SA's healthcare system, especially in terms of in-hospital treatment of severe cases. The impact of COVID-19 in Mozambique and Eswatini at the time of writing has been comparatively mild, but is increasing. It is therefore imperative to reduce as far as possible the number and rate of hospitalisations caused by trauma and other diseases, including malaria. Malaria incidence in SA is seasonal and peaks in the wetter summer months, especially during January to April.[2] Although malaria incidence in SA is currently low, the risk of outbreaks is always present, with the most recent having occurred in 2017 and, at a more localised level in Limpopo Province, in 2019. The reasons for these latest outbreaks are varied and include unusually high rainfall and cross-border movement of migrant populations, fuelling local transmission. These issues are particularly pertinent to COVID-19 in SA's malaria-affected districts. They highlight the importance of mitigating factors contributing to high malaria incidence and consequent hospitalisations, which may be further exacerbated by COVID-19/malaria coinfections and the re-opening of SA's borders with those neighbouring countries with higher malaria transmission intensities.

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.

Androgen receptor expression is associated with adverse pathological features in ureteral but not in pelvicalyceal urothelial carcinomas of the upper urinary tract.

PURPOSE: This study aims to determine the significance of androgen receptor (AR) expression in urothelial carcinoma of the upper urinary tract (UTUC). METHODS: AR expression was assessed on tissue microarrays containing specimens of 737 patients with UTUC who underwent radical nephroureterectomy with curative intent. AR expression was correlated with clinical and pathological tumor features as well as recurrence-free survival (RFS), cancer-specific survival (CSS) and overall survival (OS). RESULTS: Overall, AR was expressed in 11 % of tumors. AR expression was significantly associated with tumor necrosis as well as sessile and multifocal tumor growth but not with RFS, CSS or OS. AR was detected nearly twice as often in tumors of the ureter than of the pelvicalyceal system (p = 0.005). Subgroup analyses showed that the significant associations of AR with unfavorable pathologic features were exclusively attributable to tumors located in the ureter. However, in both ureteral and pelvicalyceal tumors, AR status was independent of RFS, CSS and OS. CONCLUSIONS: In this cohort of patients treated with RNU, AR expression was found in approximately 10 % of UTUCs, twice as often in ureteral than in pelvicalyceal tumors. While AR expression had no impact on postoperative prognosis, it was significantly associated with unfavorable pathologic features in ureteral tumors. Steroid hormone signaling might be relevant for future investigations of differences between ureteral and pelvicalyceal tumors.

Epidemiology of malaria in South Africa: from control to elimination.

Locally specific epidemiological understanding is pivotal to the success of malaria elimination in South Africa. Here, we focus on how the host, vector, parasite and environment and their interactions have influenced malaria incidence in South Africa between 1995 and 2012. Broad environmental considerations are necessary, including the physical (temperature and humidity), social (migration patterns), economic (quality of housing stock) and political (regional collaboration). 

Case management of malaria: diagnosis.

Laboratory diagnosis of malaria in South Africa has traditionally relied on microscopic examination of stained blood films. More recently, rapid diagnostic tests (RDTs) have been introduced into routine use, and molecular methods like polymerase chain reaction are useful in certain situations. As with all laboratory tests, each technique has its advantages and disadvantages. Microscopy and RDTs, if appropriately quality assured, are adequate for clinical case management. For elimination, active surveillance will need to be expanded substantially, with wider use of more sensitive diagnostic nucleic acid amplification techniques, and/or serology. To facilitate surveillance activities, techniques suitable for field or near-field use would be ideal. A long-running external quality assessment programme in South Africa has shown some deficiencies in the quality of malaria diagnosis in routine laboratories. Quality systems across the spectrum of diagnostic facilities in South Africa need strengthening, to ensure progress towards elimination. 

Case management of malaria: treatment and chemoprophylaxis.

Malaria case management is a vital component of programmatic strategies for malaria control and elimination. Malaria case management encompasses prompt and effective treatment to minimise morbidity and mortality, reduce transmission and prevent the emergence and spread of antimalarial drug resistance. Malaria is an acute illness that may progress rapidly to severe disease and death, especially in non-immune populations, if not diagnosed early and promptly treated with effective drugs. In this article, the focus is on malaria case management, addressing treatment, monitoring for parasite drug resistance, and the impact of drug resistance on treatment policies; it concludes with chemoprophylaxis and treatment strategies for malaria elimination in South Africa. 

What will move malaria control to elimination in South Africa?

In this supplement, several authors have shared lessons from the past and identified factors that led to the significant reductions in malaria morbidity and mortality during the past half-century in South Africa. In addition, strategies for achieving malaria elimination have been proposed. Here, we highlight the gaps that have been identified and make proposals for taking South Africa from malaria control to elimination.

What factors influence the results of coronary artery bypass grafting in aged patients?

AIM: Early and late results were studied in order to improve the indication for coronary artery bypass grafting (CABG) and to enhance METHODS: A total of 1 973 patients aged 70 years and older who had undergone isolated CABG were studied. Elective operations (EL) were performed in 1 716 patients and 257 patients underwent urgent or emergency operations (UR/EM). Patients were divided into two groups; 104 patients aged 80 years and older (Oct. Group) and 1 869 patients of septuagenarians (Sept. Group). There were no differences between the groups in the number of diseased vessels. RESULTS: Total operative mortality rates in the Oct. and the Sept. groups were 7% and 4%, respectively. The operative mortality of elective surgery was 4% in both groups. The operative mortality of UR/EM CABG was significantly higher in the Oct. group than in the Sept. group (21% vs 6%). Operative mortality was significantly higher in patients with preoperative poor (<49%) left ventricular ejection fraction (LVEF) than in patients with higher (>50%) LVEF (6% vs 3%). Among preoperative risk factors, diabetes mellitus and peripheral vascular disease were significant contributory factors to operative death. In the follow-up study, 70% patients of the Oct. group and 72% patients of the Sept. group survived. Preoperative number of diseased vessels and number of CABG grafts did not influence the early and late CONCLUSION: Preoperative poor LVEF, diabetes mellitus and peripheral vascular disease were significant contributory factors to operative death. When feasible, CABG in octogenarians should be performed electively.

Hemofiltration during cardiopulmonary bypass for high risk adult cardiac surgery.

AIMS: The role of hemofiltration (HF) during cardiopulmonary bypass (CPB) in adult cardiac surgery is controversial. It may be beneficial during prolonged CPB in high-risk surgery. Accordingly, we sought to compare two groups of patients undergoing high-risk cardiac surgery with or without HF. METHODS: One hundred and eighteen patients who underwent complex cardiac surgical procedures during a 12-month period were divided into two groups. Group I (n=61) comprised patients who were treated with hemofiltration during CPB. Group II (n=57) were not filtered. Estimated risk of death, standard demographic, clinical and surgical features were obtained and predetermined outcomes were studied. Statistical comparisons were made. RESULTS: Age, procedure times and mortality rates were similar in both groups. The mean volume of fluid removed in group I was 3.4 L. The preoperative mean Parsonnet score was 24.8 in group I and 22.5 in group II (ns). Postoperative serum hemoglobin, hematocrit, platelet, and albumin levels were all significantly higher in group I patients (p=0.0015) indicating hemoconcentration. Post-operative chest drainage showed a trend toward decreased post-operative bleeding in group I (p=0.065). Postoperative pleural effusions requiring chest tube drainage were significantly less in group I (9.8% vs. 29.8% 6; p = 0.0062). The incidence of lung infection was also decreased from 26.3% to 13.1% (p=0.05). Operative mortality was similar in both groups (11.4% in group 1, 10.5% in group II, ns). CONCLUSION: Hemofiltration during CPB attenuates postoperative anemia, thrombocytopenia and hypoalbuminemia, may reduce post-operative bleeding and appears to decrease post-operative pulmonary complications.