Erythromelalgia. Part II: Differential diagnoses and management.

The management of erythromelalgia is challenging and requires multidisciplinary effort. Patient education is crucial as unsafe self-administered cooling techniques can lead to significant morbidity, including acral necrosis, infection, and amputation. The goal of management is pain control, reduction of flare frequency, and prevention of complications. This text is focused on the management of erythromelalgia and several other incompletely understood and under-recognized neurovascular disorders such as red scrotum syndrome, red ear syndrome, facial flushing, and complex regional pain syndrome.

Erythromelalgia. Part I: Pathogenesis, clinical features, evaluation, and complications.

Erythromelalgia is a rare pain disorder that is underrecognized and difficult-to-treat. It is characterized by episodes of extremity erythema and pain that can be disabling; it may be genetic, related to an underlying systemic disease, or idiopathic. Considering the prominent cutaneous features characteristic of the condition, dermatologists can play an important role in early recognition and limitation of morbidity. The first article in this 2-part continuing medical education series reviews the epidemiology, pathogenesis, clinical manifestations, evaluation, and complications.

Multiple Flesh-Colored Nodules: A Patient with Multiple BAP 1-Inactivated Melanocytic Tumors.

A 22-year-old man without previously known skin disease presented with multiple asymptomatic, skin-brown to red-brown papules on the head and neck for 1 year (Figure 1). The diagnoses considered included benign intradermal or compound nevi, atypical nevi, and neurofibromas. Shave biopsies of three lesions revealed intradermal melanocytic lesions comprising large epithelioid melanocytes flanked by small banal melanocytes (Figure 2). All nevi had a low proliferation index, absent junctional component as demonstrated by a dual Ki-67/Mart-1 immunostain, and no dermal mitotic figures. Immunostaining demonstrated lesional melanocytes positive for p16, but the larger epithelioid melanocytes in these lesions lacked nuclear expression of ubiquitin carboxyl-terminal hydrolase protein (BAP-1; Figure 3). The diagnosis of a BAP-1-inactivated nevus was made, and the patient was referred for genetic counseling and screening for associated malignancies. Given that the lesions involved deep margins, the same were completely excised.

Cutaneous tuberculosis. Part I: Pathogenesis, classification, and clinical features.

Tuberculosis is an ancient disease that continues to affect an estimated 10 million people per year and is responsible for 1.4 million deaths per year. Additionally, the HIV epidemic and multidrug resistance present challenges to disease control. Cutaneous tuberculosis is an uncommon, often indolent, manifestation of mycobacterial infection that has a varied presentation. Its diagnosis is challenging, as lesions mimic other, more common conditions and microbiological confirmation is often not possible. Cutaneous tuberculosis can be broadly categorized into multibacillary and paucibacillary forms. Approximately one-third of skin tuberculosis is associated with systemic involvement. By recognizing cutaneous tuberculosis early, dermatologists can play an important role in disease control. The first article in this 2-part continuing medical education series describes the latest epidemiology, microbiology, and pathogenesis of tuberculosis. Furthermore, we review the classification, clinical manifestations, common clinical differentials, and systemic involvement that occur in cutaneous tuberculosis.

Cutaneous tuberculosis. Part II: Complications, diagnostic workup, histopathologic features, and treatment.

Despite the availability of effective treatment regimens for cutaneous tuberculosis, challenges to disease control result from delayed diagnosis, infection with multidrug-resistant mycobacterial strains, and coinfection with HIV. Delayed diagnosis can be mitigated when dermatologists are sensitized to the clinical signs and symptoms of infection and by the incorporation of appropriate diagnostic tests. All cases of cutaneous tuberculosis should be confirmed with histopathology and culture with or without molecular testing. In each case, a thorough evaluation for systemic involvement is necessary. Mycobacteria may not be isolated from cutaneous tuberculosis lesions and therefore, a trial of antituberculosis treatment may be required to confirm the diagnosis. The second article in this 2-part continuing medical education series describes the sequelae, histopathology, and treatment of tuberculosis.

Cutaneous adverse events of immune checkpoint inhibitor therapy: incidence and types of reactive dermatoses.

BACKGROUND: Dermatoses are common and potentially serious complications of programmed cell death receptor PD-1 immune checkpoint inhibitor (anti-PD-1 ICI) therapy. Understanding their incidence is necessary to support clinical awareness, diagnosis, and management. OBJECTIVE: To examine the incidence and odds of reported non-cancerous dermatoses in the setting of anti-PD-1 ICI therapy. METHODS: Cross-sectional study of anti-PD-1 (pembrolizumab or nivolumab) treated patients at a tertiary healthcare institution. Selected dermatologic events following immunotherapy were identified in the electronic medical record. Comparator arm were patients that developed these same dermatoses without receiving anti-PD-1 ICI therapy. RESULTS: There were 13.7% (254/1857) patients that developed one of 28 dermatoses. Compared with the general population, patients treated with anti-PD-1 had a greater risk for development of mucositis (OR 65.7, 95% CI 35.0-123.3), xerostomia (OR 11.9, 95% CI 8.4-16.8), pruritus (11.3, 95% CI 8.9-14.3), and lichen planus/lichenoid dermatitis (OR 10.7, 95% CI 5.6-20.7). CONCLUSIONS: We report the frequency of dermatoses encountered in the setting of ICI therapy, both common (pruritus, rash, vitiligo) and uncommon (scleroderma, urticaria).

A Brief Overview of Recurrent Pericarditis Management and the Potential of Rilonacept as a New Therapeutic Option.

Recurrent pericarditis affects 15-30% of patients after acute pericarditis. A large number of the patients with recurrent pericarditis can become corticosteroid dependent, leading to disease chronicity and drug dependence, with additional morbidity from long-term steroid use. Recent randomized trials indicate the efficacy of the interleukin-1 inhibitors anakinra and rilonacept in recurrent pericarditis, including colchicine-resistant and corticosteroid-dependent cases. In particular, rilonacept was assessed in the RHAPSODY clinical trial and found to be a potential treatment option that would decrease recurrent episodes, enabling patients to be weaned off steroids. Additionally, new data indicate that rilonacept should be considered as an option for patients with recurrent pericarditis, as add-on therapy to colchicine and nonsteroidal anti-inflammatory drugs, in place of steroids. We review the current management options for recurrent pericarditis as well as rilonacept as a prospective new addition to our armamentarium.

COVID-19 and Healthcare Worker: What We Need to Know.

COVID-19 pandemic has challenged and overwhelmed most healthcare institutions and healthcare workers, across the world. Despite being unprepared for this pandemic, frontline workers have worked relentlessly to provide the much-needed care to these patients. Doctors from different branches of medicine, including dermatologists, came forward and played a substantial role in mitigating the impact of this pandemic on the general population. Sadly, in the process, these healthcare workers faced many personal, social, psychological, economic, and health-related issues. The psychological burden and health-related issues received due attention in the main-stream news as well as scientific research papers. With most frontline workers isolated from their families, social media became the new platform to reduce the sense of isolation and share their anxiety, insomnia, and fatigue. This article is aimed at highlighting various challenges faced by healthcare workers during the ongoing COVID-19 pandemic.

Ruptured Sinus of Valsalva Aneurysm: Use of Multimodality Imaging in Delineating Structure and Function.

Sinus of Valsalva aneurysm is a rare defect that can present with fatal complications in case of rupture. Heart failure symptoms are common due to high fraction of the resultant shunt. Multimodality imaging and invasive hemodynamic assessment is essential for comprehensive evaluation of the defect and guiding surgical planning. We describe the case of a 40-year-old woman who presented with heart failure symptoms and was found to have ruptured sinus of Valsalva aneurysm on transthoracic echocardiogram. Cardiac computed tomography angiography further characterized the defect and the associated anomalies. Right heart catheterization assessed the hemodynamic significance of the left to right shunt. Intraoperative findings highlighted the associated congenital anomalies including supracristal ventricular septal defect. The use of intraoperative transesophageal echocardiography proved essential in detecting worsening of the right ventricular outflow track infundibular dynamic obstruction post repair thus delineating the importance of maintaining adequate cardiac preload. This case highlights a stepwise approach in the anatomical characterization of sinus of Valsalva aneurysm using multimodality imaging and the use of hemodynamic assessment and intraoperative imaging to guide surgical planning.

Psoriasis does not worsen outcomes in patients admitted for ischemic stroke: an analysis of the National Inpatient Sample.

Psoriasis is a chronic inflammatory state associated with an increased risk of cardiometabolic diseases, stroke, and mortality. Although psoriasis increases the risk of ischemic stroke, whether outcomes, including mortality, are adversely affected is unknown.This study aims to compare inpatient mortality of patients admitted for ischemic stroke with and without psoriasis. The secondary outcome measures were hospital length of stay (LOS), total hospital charges, odds of receiving tissue plasminogen activator (TPA), and mechanical thrombectomy between both groups.Data were obtained from the National Inpatient Sample (NIS) 2016 and 2017 databases using the International Classification of Diseases, Tenth Revision, Clinical Modification codes. Multivariable logistic and linear regression analysis were used accordingly to account for confounders of the outcomes.The combined 2016 and 2017 NIS database comprised over 71 million discharges. Of these, ischemic stroke accounted for 525,570 hospitalizations and 2425 (0.5%) had a concomitant diagnosis of psoriasis. Patients hospitalized for ischemic stroke with coexisting psoriasis did not have a difference in inpatient mortality (3.5% vs 5.5%; p=0.285) compared with those without psoriasis. However, psoriasis cohort had shorter LOS (5.0 vs 5.7 days; p=0.029) and lower total hospital charges ($60,471 vs $70,246; p=0.003) compared with the non-psoriasis cohort. The odds of receiving TPA and undergoing mechanical thrombectomy were not different in both groups.Inpatient mortality, odds of receiving TPA, and undergoing mechanical thrombectomy in patients who had an ischemic stroke with or without psoriasis were not different. However, patients with psoriasis had a significantly shorter LOS and lower hospital charges.

Gout Pharmacotherapy in Cardiovascular Diseases: A Review of Utility and Outcomes.

Hyperuricemia and gout have been linked to an increased risk for cardiovascular (CV) disease, stroke, hypertension, heart failure, and chronic kidney disease, possibly through a proinflammatory milieu. However, not all the drugs used in gout treatment improve CV outcomes; colchicine has shown improved CV outcomes in patients with recent myocardial infarction and stable coronary artery disease independent of lipid-lowering effects. There is resurging interest in colchicine following publication of the COLCOT, LoDoCo, LoDoCo2, LoDoCo-MI trials, and COLCORONA trial which will shed light on its utility in COVID-19. Our aim is to review the CV use of colchicine beyond pericardial diseases, as well as CV outcomes of the available gout therapies, including allopurinol and febuxostat. The CARES trial and its surrounding controversies, which lead to the US FDA 'black box' warning on febuxostat, in addition to the recent FAST trial which contradicts this and finds febuxostat to be non-inferior, are discussed in this paper.