Neonatal MIS-C: Managing the Cytokine Storm.

A term infant girl with uneventful antenatal history had an erythematous rash followed by fever from day 8. She was diagnosed with late-onset sepsis and was treated accordingly. She received immunoglobulin for persistent thrombocytopenia, after which there was transient improvement.

Sarcoid Heart Disease: Review of Current Knowledge.

Sarcoidosis is a granulomatous disease with the potential of multiple organ system involvement and its etiology remains unknown. Cardiac involvement is associated with worse clinical outcome, and has been reported to be 20-30% in white and as high as 58% in Japanese populations with sarcoidosis. Clinical manifestations of cardiac sarcoidosis highly depend on the extent and location of granulomatous inflammation.

Reversible right-sided heart failure and pulmonary hypertension caused by scurvy in a 7-year-old boy with autism spectrum disorder and a review of the literature.

Scurvy, a condition caused by vitamin C deficiency, is rare, especially in high-income countries. Symptoms of scurvy are typically characterised by dermatological disorders such as poor wound healing and tooth loss, but there is not usually cardiac involvement. A case of reversible pulmonary hypertension and right-sided heart failure owing to scurvy in a 7-year-old boy with autism spectrum disorder is reported.

Case Report: First Two Identified Cases of Fabry Disease in Central Asia.

Fabry disease (FD, OMIM #301500) is a rare, progressive, X-linked inherited, genetic disease due to the functional deficiency of lysosomal α-galactosidase (α-GAL) that leads to the accumulation of glycosphingolipids (mainly globotriaosylceramide or Gb) and its derivative globotriaosylsphingosine or lyso-Gb. Classic FD is a multisystem disorder which initially presents in childhood with neuropathic pain and dermatological, gastrointestinal, ocular, and cochleo-vestibular manifestations. Over time, end-organ damage such as renal failure, cardiac arrhythmia and early stroke may develop leading to reduced life expectancy in the absence of specific treatment.

Skin and sepsis: contribution of dermatology to a rapid diagnosis.

In patients who develop sepsis, whether due to primary, secondary or metastatic lesions, the skin is frequently affected. However, there are unresolved aspects regarding the general clinical manifestations in the skin or the prognosis and/or therapeutic implications. The main challenge in the approach to sepsis is its early diagnosis and management.

Atypical Manifestations of Severe Acute Respiratory Syndrome Coronavirus 2 Infection in Children: A Systematic Review.

Background: In December 2019, a local outbreak of pneumonia was presented in Wuhan (China) and quickly identified to be caused by a novel coronavirus, Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2). The disease caused by SARS-CoV-2 was named COVID-19 and was soon declared a pandemic because of the millions of infections and thousands of deaths worldwide. Children infected with SARS-CoV-2 usually develop the asymptomatic or mild type of disease compared to adults.

Comparative Analysis of BTK Inhibitors and Mechanisms Underlying Adverse Effects.

The cytoplasmic protein-tyrosine kinase BTK plays an essential role for differentiation and survival of B-lineage cells and, hence, represents a suitable drug target. The number of BTK inhibitors (BTKis) in the clinic has increased considerably and currently amounts to at least 22. First-in-class was ibrutinib, an irreversible binder forming a covalent bond to a cysteine in the catalytic region of the kinase, for which we have identified 228 active trials listed at ClinicalTrials.

Factors linked to severe outcomes in multisystem inflammatory syndrome in children (MIS-C) in the USA: a retrospective surveillance study.

Background: Multisystem inflammatory syndrome in children (MIS-C) is a newly identified and serious health condition associated with SARS-CoV-2 infection. Clinical manifestations vary widely among patients with MIS-C, and the aim of this study was to investigate factors associated with severe outcomes.

Methods: In this retrospective surveillance study, patients who met the US Centers for Disease Control and Prevention (CDC) case definition for MIS-C (younger than 21 years, fever, laboratory evidence of inflammation, admitted to hospital, multisystem [≥2] organ involvement [cardiac, renal, respiratory, haematological, gastrointestinal, dermatological, or neurological], no alternative plausible diagnosis, and either laboratory confirmation of SARS-CoV-2 infection by RT-PCR, serology, or antigen test, or known COVID-19 exposure within 4 weeks before symptom onset) were reported from state and local health departments to the CDC using standard case-report forms.

Dermatological manifestations during COVID-19 infection: a case series and discussion on the problem of differential diagnosis.

On March 11, 2019 the World Health Organization (WHO) declared Coronavirus disease-2019 (COVID-19), caused by SARS-CoV-2, as a pandemic. As of 15/01/2021, more than ninety million cases of infections have been confirmed, with almost two million related deaths. SARS-CoV-2 causes bilateral interstitial pneumonia, which can responsible of respiratory failure in the most severe cases, but the virus has also a wide range of other manifestations, including gastrointestinal, cardiovascular, neurological, and cutaneous signs and symptoms.

A rare case of scrub typhus vasculitis presenting as acute coronary syndrome diagnosed by skin manifestations.

A 48-year-old diabetic man presented with complaints of acute onset chest heaviness with palpitations, anxiety and headache. He had raised troponin-T level and electrocardiogram showed ST elevation myocardial infarction. There was a prior history of fever of 4 days duration with associated abdominal pain.

Cardiac manifestations of eosinophilic granulomatosis with polyangiitis from a single-center cohort in China: clinical features and associated factors.

Background: Cardiac manifestations are common and life-threatening in eosinophilic granulomatosis with polyangiitis (EGPA), which remains poorly studied in China. We aim to investigate its clinical features, associated factors, treatment, and outcomes.

Methods: We reviewed the clinical records of 110 EGPA patients and examined the independent factors associated with cardiac manifestations using multivariate logistic regression.

Concomitant Large Loculated Pleural and Pericardial Effusions in a Patient with Rheumatoid Arthritis on Methotrexate.

Rheumatoid arthritis (RA) is the most common multisystemic autoimmune inflammatory joint disorder, affecting nearly 1.3 million adults in the US. RA has high economic and social burdens.

Kawasaki disease for the paediatric dermatologist: skin manifestations and new insights into the pathophysiology.

Early diagnosis of Kawasaki disease (KD) is critical to allow prompt initiation of treatment and avoid cardiac complications. All children with KD have fever accompanied by clinical signs, with four of the five classic criteria for complete KD being mucocutaneous, thus creating an important role for dermatologists. Moreover, dermatologists must be familiar with other dermatological findings that are not included in the American Heart Association classification criteria but can support the diagnosis, particularly in incomplete forms of the disease.

Cogan syndrome: An autoimmune eye and ear disease with systemic manifestations.

Cogan syndrome (CS) is a rare vasculitis seen primarily in young adults. It predominantly affects eyes, ears and the heart with characteristic findings of interstitial keratitis, sensorineural hearing loss and vestibular dysfunction. A high index of suspicion is required to diagnose this rare disorder.

Massive gingival bleed: a rare manifestation of cyclosporine toxicity.

A 12-year-old patient of thalassaemia major developed autoimmune cytopaenia after undergoing haematopoietic stem cell transplantation. She was started on cyclosporine (CsA) in view of poor response to steroids. She developed CsA toxicity manifesting as gum hyperplasia with multiple episodes of gum bleed.

Dapsone-induced DRESS after infliximab-induced vasculitis: a case of cerebral infarction in the context of multiple drug reactions.

Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a life-threatening condition characterised by peripheral eosinophilia, rash and multi-organ failure arising several weeks after exposure to the culprit medication. Although rare, DRESS syndrome triggered by specific agents has been associated with specific genetic polymorphisms more prevalent in different ethnic groups, including an association between dapsone-induced DRESS and Human Leukocyte Antigen (HLA)-B:13*01, a single nucleotide polymorphism more prevalent in those of Asian descent. DRESS and drug-related vasculitis may affect any organ system including the central nervous system (CNS), usually manifesting as encephalitis, meningitis or embolic cerebrovascular accidents related to eosinophilic cardiac disease and thrombosis.

Neonatal lupus presenting as a non-specific rash in primary care.

Neonatal lupus erythematosus is a rare autoimmune disease caused by passive transplacental acquisition of maternal autoantibodies manifesting in cutaneous, cardiac, haematological and hepatobiliary abnormalities. The hallmark dermatological finding is erythematous annular lesions with a predilection for photo-exposed areas of the skin. We present a case of a female infant born to a mother with Sjogren's syndrome, who initially presented to an ambulatory care setting with non-specific erythematous papules involving the face and scalp.

Characteristics and Outcomes of Coronary Artery Involvement in Polyarteritis Nodosa.

Background: Coronary artery involvement is a severe but uncommon manifestation of polyarteritis nodosa (PAN), so clinicians have little knowledge of it. Our aim was to investigate the clinical characteristics, risk factors and outcomes of patients with PAN complicated with coronary artery lesions.

Methods: Data from 145 patients with PAN who were admitted to Peking Union Medical College Hospital from January 2000 to September 2019 were retrospectively collected.

Multisystem Imaging Manifestations of COVID-19, Part 2: From Cardiac Complications to Pediatric Manifestations.

Infection with severe acute respiratory syndrome coronavirus 2 results in coronavirus disease 2019 (COVID-19), which was declared an official pandemic by the World Health Organization on March 11, 2020. COVID-19 has been reported in most countries, and as of August 15, 2020, there have been over 21 million cases of COVID-19 reported worldwide, with over 800 000 COVID-19-associated deaths. Although COVID-19 predominantly affects the respiratory system, it has become apparent that many other organ systems can also be involved.

Multisystem Imaging Manifestations of COVID-19, Part 1: Viral Pathogenesis and Pulmonary and Vascular System Complications.

Infection with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) results in coronavirus disease 2019 (COVID-19), which was declared an official pandemic by the World Health Organization on March 11, 2020. The infection has been reported in most countries around the world. As of August 2020, there have been over 21 million cases of COVID-19 reported worldwide, with over 800 000 COVID-19-associated deaths.

Clinical Characteristics, Treatment, and Outcomes in Patients with Idiopathic Inflammatory Myopathy Concomitant with Heart Failure.

This study sought to evaluate clinical features, treatment patterns, and outcomes of patients with idiopathic inflammatory myopathy (IIM) complicated by heart failure (HF). Thirty-two patients with IIM-HF admitted to the Peking Union Medical College Hospital between January 1999 and January 2018 were retrospectively reviewed, including 14 patients with polymyositis, 11 with dermatomyositis, and 7 with overlap syndrome. Survivors and no-survivors were compared on clinical characteristics and treatment.

Toxicity of chloroquine and hydroxychloroquine following therapeutic use or overdose.

Introduction: While chloroquine, a derivative of quinine, has been used as an antimalarial for 70 years, hydroxychloroquine is now used to treat conditions such as rheumatoid arthritis and systemic lupus erythematosus. In 2020, hydroxychloroquine (and to a lesser extent chloroquine) also received attention as a possible treatment for severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2). During investigation for treating coronavirus disease 2019 (COVID-19) caused by SARS-CoV-2, concerns for serious adverse events arose.

The emergence of targetable MEKanisms in sporadic lymphatic disorders.

Sporadic lymphatic diseases are orphans among orphans in the medical community, a diverse collection of disorders at the intersection of cardiac, gastrointestinal, pulmonary, dermatologic, and oncologic disease that receives only passing attention in medical school and that no subspecialty in medicine fully embraces as its own. They often present in a confusing and illusive manner, with a fractured bone, expectoration of blood or a branching airway cast, a swollen limb or a collection of chylous material; protean manifestations that can challenge even the most expert diagnostician. Yet many of these acquired disorders have been discovered to have a targetable genetic basis, and as the case report of Foster et al (2020) demonstrates, the sedulous clinician-patient dyad can be rewarded with an almost miraculous result when the molecular pathogenesis of the disease is pursued and an exquisitely targeted therapy is administered.

Cardiac involvement in adult-onset Still's disease: Manifestations, treatments and outcomes in a retrospective study of 28 patients.

Objective: Adult-onset Still's disease (AOSD) is a rare inflammatory disease that may be life-threatening if complicated by cardiac problems. We performed a retrospective multicenter study to describe the manifestations, treatments and outcomes of cardiac involvement in AOSD.

Methods: We reviewed the medical databases of eight centers.