Strawberry Tongue Research Articles

The appearance of oral manifestations in children infected with COVID-19: A critical review of the literature

Objective The aim of this narrative review of the literature is to summarize the evidence on the prevalence of oral manifestations and to ascertain at what point in time oral manifestations present in children infected with SARS-CoV-2. Method Databases PUBMED, Google Scholar and Ebsco were used. The search terms: COVID 19, oral manifestations and child, along with age filters were applied to generate the articles published between 2020-2021.The abstracts of the articles were first read and then those articles that were relevant were evaluated in detail. Results Hyperaemic pharynx and dysgeusia were prevalent in children with COVID-19 disease while edematous and erythematous lips and strawberry tongue were frequently seen in patients diagnosed with multisytem inflammatory syndrome in children. In some sources the onset of oral manifestations was unclear resulting in inconclusive evidence. Conclusion Oral manifestations are commonly seen amongst the earliest manifestations of COVID-19, and may happen prior to presenting other symptoms, or up to a week after other manifestations of the disease. The appearance of oral manifestations in children with COVID -19 like symptoms may be an indicator of disease severity and the likelihood that disease progression could potentially be more severe and have more detrimental outcomes.

Kawasaki Disease Complicated with Giant Coronary Aneurysm in Case of Systematized Epidermal Nevus

Kawasaki disease (KD) is an acute systemic vasculitis that involves coronary arteries. Systematized epidermal nevus is a skin disease characterized by extensive verrucous and hyperchromic plaques. A male patient, previously diagnosed with systematized epidermal nevus, was referred for high fever. Eye injection, red lip, strawberry tongue, and enlarged neck node were accompanied. Intravenous immunoglobulin (IVGG) and high-dose aspirin were given for the treatment of KD. An echocardiogram revealed a small aneurysm in the right coronary artery (RCA). Second IVGG was administered because of refractoriness. The size of the aneurysm was increased to medium in RCA and another medium-sized aneurysm was found in the left anterior descending artery (LAD). Clopidogrel was added. After 3 days of defervescence, he was discharged. Warfarin was added for a giant aneurysm in LAD, aggravated 8 weeks later after the presentation. 11 months later, giant aneurysm was changed to medium sized aneurysm. Patient is at following with dual antiplatelet therapy without warfarin.

Incomplete Kawasaki Disease Presenting as Retropharyngeal Abscess: A Case Report and Review of the Literature.

Kawasaki disease (KD) is a cryptic and self-limiting vasculitis predominantly seen in children, often posing a diagnostic challenge due to its varied clinical presentations. Among these, the emergence of deep neck infections, particularly retropharyngeal abscesses, stands out as an extremely rare manifestation. Herein, we present a case of a 10-year-old girl who was hospitalized for fever, neck pain, swollen cervical lymph nodes, and increased inflammation markers. Although anti-infective treatment was initiated, her condition remained unchanged. A cervical computed tomography scan revealed an abscess in the retropharyngeal space. Remarkably, on the third day post-admission, the patient developed symptoms synonymous with KD, such as conjunctival redness, reddened lips, and a strawberry tongue. Subsequent treatment with high-dose intravenous immunoglobulins (IVIG) and oral aspirin led to swift symptom relief, including complete abscess resolution verified by a follow-up neck magnetic resonance imaging. This unique co-presentation of KD and a retropharyngeal abscess, possibly linked to infections like Streptococcus or Staphylococcus aureus, underscores the importance of quick diagnosis and KD management, especially when conventional treatments prove ineffective.

Dilemma in diagnosing incomplete Kawasaki disease in a resource limited setting

Introduction and importance: Kawasaki disease (KD) is an acute febrile systemic vasculitis that predominantly affects small to medium sized vessels and mostly occurs in children below 5 years of age. The morbidity and mortality mostly occur due to cardiac involvement. Case presentation: The authors present a case of a 5-year-old male child from hilly region of Nepal who presented with fever for 7 days along with strawberry tongue and non-exudative conjunctivitis without rashes, extremity changes or lymphadenopathy. A suspicion of incomplete KD (IKD) was made. The notable investigation findings were increased erythrocyte sedimentation rate, C-reactive protein, leucocyte count and platelets. Echocardiography showed normal findings. Based on the clinical features and supplemental laboratory findings, a diagnosis of IKD was made. The patient improved after intravenous immunoglobulin and Aspirin. Clinical discussion: The main learning objective that the authors get from this case is the challenges in the diagnosis of IKD in the resource limited setting like Nepal. Whether or not to start intravenous immunoglobulin is a dilemma for the physician in most of the cases of suspected IKD, due to the high cost and poor availability of intravenous immunoglobulin in this setting. Hence, the use of inflammatory markers, supplemental laboratory findings together with the few diagnostic criteria met by the patient helps in making a diagnosis and institute timely treatment with intravenous immunoglobulin and aspirin. Conclusion: Diagnosis of KD in difficult in resource limited setting.

Clinical manifestations and risk factors of shock in children with multisystem inflammatory syndrome

Multisystem inflammatory syndrome in children (MIS-C) is a novel disease associated with COVID-19. The COVID-19 epidemic peaked in May 2022 in Taiwan, and we encountered our first case of MIS-C in late May 2022. We aimed to present patients' clinical manifestations and identify risk factors for shock. We included patients diagnosed with MIS-C at two medical centers from May 2022 to August 2022. We separated those patients into two groups according to whether they experienced shock. We collected demographic, clinical manifestation, and laboratory data of the patients and performed statistical analysis between the two groups. We enrolled 28 patients, including 13 (46%) with shock and 15 (54%) without shock. The median age was 6.4 years (IQR: 1.9-7.5). In single variable analysis, patients with shock tended to be older, had more neurological symptoms, more conjunctivitis and strawberry tongue, lower lymphocyte count, lower platelet counts, and higher C-reactive protein, higher procalcitonin, higher ferritin, and higher D-dimer levels than those without shock. The area under the ROC curve that used procalcitonin to be the risk factor of shock with MIS-C was 0.815 (95% CI 0.644 to 0.987). The cutoff value obtained by ROC analysis of procalcitonin was 1.68ng/mL. With this cutoff, the test characteristics of procalcitonin were as follows: sensitivity 77%, specificity 93%, positive predictive value 91%, negative predictive value 82%. Multivariable analysis revealed that procalcitonin was the only independent risk factor of shock with MIS-C on admission (OR, 26.00, 95% CI, 1.01-668.89). MIS-C patients with high initial procalcitonin levels have higher risks of experiencing shock and may need ICU admission.

Anakinra and hepatotoxicity in pediatric rheumatology: a case series

BackgroundAnakinra is a recombinant interleukin-1 (IL-1) receptor antagonist used in systemic juvenile idiopathic arthritis (sJIA), refractory Kawasaki disease (KD) and cryopyrin-associated autoinflammatory syndrome (CAPS). Anakinra associated hepatotoxicity, while rare, has been described in several cases in daily practice. ​In this case series the authors describe three pediatric patients with this side effect in the setting of severe macrophage activation syndrome (MAS) in KD and sJIA.Case presentationThe first patient was a 12-year-old boy who presented with fever, maculo-papular exanthema and polyarthralgia. Tonsillitis, distal limb induration and tender cervical lymph nodes were observed. Erythrocyte-sedimentation rate (ESR), C-reactive protein (CRP), ferritin (11,975 ng/mL), D-dimers (5,98 mg/L FEU) and soluble CD25 (3645 pg/mL) levels were elevated. Exclusion of sepsis / toxic shock syndrome warranted introduction of IV methylprednisolone and immunoglobulin (IG IV), with partial response. A MAS secondary to KD was assumed, and anakinra 2 mg/kg/day was introduced. Twenty days later he developed new-onset nausea and severe cyto-cholestasis, normalizing after 2 months of drug discontinuation. Posterior onset of polyarthritis and evanescent lead to a final diagnosis of sJIA. The second patient was a 2-year-old boy with a 10-day history of fevers, generalized rash, hepatosplenomegaly and strawberry tongue. Leucocytosis with neutrophilia and elevated CRP were observed. Initial treatment with IVIG in the setting of incomplete KD was ineffective. Mild anaemia, leukopenia and very high serum ferritin (maximum 26,128 ng/mL) ensued. Presumptive sJIA associated MAS was treated with IV methylprednisolone and anakinra 2 mg/kg/day, with prompt response. Four weeks later transaminitis was detected, and temporary anakinra suspension led to normalisation of laboratorial values. The third case related to a 4-year-old boy presenting with fever, maculopapular rash and cervical lymphadenopathy. CRP and ESR were elevated, and KD was diagnosed. IVIG and methylprednisolone were initiated with clinical worsening, warranting for anakinra introduction at 2 mg/kg/day. After three weeks, liver enzymes progressively elevated, resolving on 2 weeks of anakinra discontinuation.ConclusionsTo the best of our knowledge, this is the first case series describing anakinra associated hepatotoxicity in pediatric patients with rheumatic diseases other than sJIA, bringing additional insight to therapeutic monitoring in patients undergoing this treatment.

A Rare Case of TMP-SMX Induced DRESS and Clinical TSS in a Pediatric Patient

Drug Reaction with Eosinophilia and Systemic Symptoms (DRESS) is a rare and potentially life-threatening adverse drug reaction. We present a case of a 14-year-old girl with overlapping DRESS syndrome and Toxic Shock Syndrome (TSS) following Trimethoprim/Sulfamethoxazole treatment. The patient developed a generalized pruritic maculopapular rash, lymphadenopathy, fever, and facial swelling. Laboratory results showed leukocytosis, elevated liver enzymes, and C-reactive protein (CRP). Despite initial treatment with methylprednisolone, her condition worsened, necessitating a transfer to a higher level of care. She exhibited fever, hypotension, tachycardia, tachypnea, strawberry tongue, and perioral impetigo, consistent with TSS. Antibiotic therapy was initiated, but eosinophilia and an abdominal desquamative rash emerged, indicative of DRESS syndrome. Treatment involved discontinuing antibiotics, administering intravenous solumedrol for possible DRESS, and restarting antibiotics due to persistent TSS features. The patient’s symptoms improved, and she was discharged on tapering steroids and antibiotics. This case highlights the coexistence of DRESS and TSS in a pediatric patient and emphasizes the importance of promptly discontinuing the offending agent and appropriate management with steroids and antibiotics. Of the participants, 65.9% reported decreased activity levels, while 46.3% experienced weight gain. Notably, 64.2% of participants had an elevated risk score, signifying an increased vulnerability to T2D.

A case of Multisystem Inflammatory Syndrome in Children (MIS-C)

Multisystem inflammatory syndrome in children (MIS-C) is a systemic disorder involving persistent fever, extreme inflammation and organ dysfunction, which is temporally associated with exposure to COVID-19. onset may be delayed or contemporary with ongoing SARS-CoV-2 infection. Here we present a case of 4-year-old girl, who was presented with high grade fever, bilateral non-purulent conjunctivitis, generalized macular rash, dry and cracked lips with strawberry tongue, abdominal pain and diarrhoea, having contact with patient of COVID-19. She had neutrophilic leukocytosis with thrombocytosis and very high inflammatory markers, CXR demonstrated bilateral peripheral patchy opacities, RT-PCR for COVID-19 was negative. According to diagnostic criteria this patient was diagnosed as Multisystem inflammatory syndrome in children (MIS-C) which is temporally associated with COVID-19 and treated successfully.
 AKMMC J 2021; 12(1) : 62-65

Intravenous immunoglobulin for the treatment of Kawasaki disease.

Intravenous immunoglobulin for the treatment of Kawasaki disease.

Nowadays a New MIS-C Mimicker: Group A Streptococcal Infections.

To the Editors: We show severe streptococcal infections in this report that mimicked the multisystem inflammatory syndrome in children (MIS-C) clinical presentation. At the start of December 2022, the cases were concurrently admitted to our hospital. We would want to share our study considering the Streptococcus pyogenes-related death cases that have recently been reported in many parts of Europe. An 8-year-old female patient complained of chest pain, headaches and fever for 3 days. The patient exhibited a maculopapular rash on the trunk, strawberry tongue, bilateral conjunctivitis, tonsillar hyperemia and cervical lymphadenopathy. Acute phase reactants were noticeably greater and acute renal failure was found in addition to the patient’s lymphopenia (C-reactive protein: 503 mg/L, procalcitonin: 49 μg/L, leucocyte count: 14.700 × 103/μL, lymphocyte count: 100 × 103/μL). The patient was receiving inotropic treatment because of hypotension. A prediagnosis of septic shock and MIS-C was made, and she was monitored in the critical care unit. Treatments with cefotaxime and clindamycin began. Intravenous immunoglobulin (IVIG) (1 g/kg/d for 2 days) and steroid (2 mg/kg/d) treatments were used when the SARS-CoV-2 antibodies were positive. The echocardiogram was normal. Streptococcus pyogenes was produced in the case’s blood culture during the follow-up, confirming the diagnosis of toxic shock syndrome. A 10-year-old female patient presented with complaints of fever, headache, vomiting and weakness for 2 days. The patient with weakness appearance had hyperemic tonsils and bilateral conjunctivitis. In the laboratory results at admission, lymphopenia and increased B-type natriuretic peptide were evident. In the acute phase reactants, the procalcitonin level was 3.7 μg/L and the CRP level was 41 mg/L. The patient had multiorgan involvement, which included the Centers for Disease Control and Prevention case description, thus treatment for MIS-C was initiated in addition to antibiotherapy even though there had been no appreciable increase in inflammatory markers. The patient showed clinical improvement within hours after fluid resuscitation and antibiotic therapy. Influenza was detected in the respiratory viral panel. Upon isolation of Streptococcus pyogenes in the throat culture during the follow-up, the diagnosis was finalized and MIS-C treatment was discontinued and targeted antibiotherapy was continued. This report was presented to highlight severe streptococcal infections mimicking MIS-C. Today, MIS-C, which is characterized by fever, elevated inflammatory markers and multisystem organ involvement, is seen in children following SARS-CoV-2 infection.1 MIS-C is an excluding diagnosis, careful consideration should go into the differential diagnosis. Given that it is overlapped with other prevalent diseases, it is a challenging process for the doctor to treat. In instances with fever, organ involvement and elevated inflammatory markers, empirical antibiotic therapy for potential bacterial agents should be started. In fact, if clinical suspicion is high, antibiotic therapy may be extended. By carefully observing the outcomes of the culture, antibiotherapy can be terminated early in instances where it is not necessary. Viral infections, such as influenza, facilitate the development of group a streptococcal infection, and this increase may have occurred due to the influenza epidemic in the northern hemisphere. The presence of SARS-CoV-2 antibodies in our case with streptococcal toxic shock syndrome increased the suspicion of MIS-C. However, it remains unclear how the antibody test should be interpreted. Because of the increasing prevalence of infection in the community, the usefulness of SARS-CoV-2 antibody testing seems to be limited.2 Therefore, cases with suspected MIS-C should be evaluated from a broad perspective in terms of bacterial infections. Delayed administration of antibiotics may result in mortality.

PS-C05-8: ERYTHROCYTE SEDIMENTATION RATE LEVELS AND ITS ASSOCIATION WITH CORONARY ARTERY INVOLVEMENT IN KAWASAKI DISEASE

Objectives: The general objective of this study is to determine association of ESR with cardiovascular involvement in Kawasaki Disease (KD) patients. Specific Objectives: Design and method: This is a cross sectional study conducted at National Childrens Hospital (NCH), a tertiary pediatric hospital. Data was gathered using charts from the medical records of patients diagnosed with KD. Study population included patients diagnosed with KD with classical presentation from year 2013–2018. The diagnostic criteria for KD require presence of fever for five days or more and at least four of five of the following: bilateral conjunctival infection without exudates, polymorphous exanthema, strawberry tongue, desquamation of palms and soles, and cervical lymphadenopathy. ESR levels were recorded both as a numerical variable and categorical variable based on the level (below 80 mm/hr, more than 80 mm/hr). Patients with coronary artery involvement included perivascular brightness, coronary artery dilatation, or coronary aneurysm on two-dimensional echocardiography done during admission for KD. Descriptive statistics was done using frequency and percentages for categorical variables, and standard deviations for continuous variables was calculated to describe the profile of the respondents. Determination of the association between ESR and CAD among KD patients was analyzed using Chi square test and stratified analysis. Odds ratio and the 95% confidence interval was also calculated. Significance was set at p value of < 0.05. Results: Of the 116 patients, 72.4% of the population were noted to have cardiac involvement and 50% had coronary artery involvement. The most common coronary artery involvement was on the proximal right coronary artery. Moreover, pericardial effusion and valvular regurgitation mostly involving the mitral valve were the most common cardiac involvement. In the population, 61 (52.6%) were male, and 26 (42.6%) of which developed CAD however no correlation between sex and CAD was revealed. Conclusion: This study showed no correlation between the level of ESR and the development of CAD. It is not recommended to obtain ESR in patients who qualify as complete KD. Hence the study does not support the practice of obtaining ESR upon admission of KD patients. Further studies on the association of ESR with CAD with higher sample size and adjusting for other confounding variables are needed.

Clinical and Laboratory Evaluations of Patients Diagnosed as Having Multisystem Inflammatory Syndrome Associated with Coronavirus Disease 2019 in Children: A Single Center Experience from Konya

Abstract Objective Multisystem inflammatory syndrome in children (MIS-C), characterized by fever, inflammation, and multiorgan dysfunction, was newly defined after severe acute respiratory syndrome coronavirus 2 infection. The clinical spectrum of MIS-C can be classified as mild, moderate, and severe. We aimed to evaluate demographics, clinical presentations, laboratory findings, and treatment modalities of patients with MIS-C according to clinical severity. Methods We performed a retrospective study of patients who were diagnosed as having MIS-C between September 2020 and October 2021 in the Necmettin Erbakan University Meram Faculty of Medicine, Türkiye. Results A total of 48 patients (24 females and 24 males) with a median age at diagnosis of 10.3 years (range: 42 months–17 years) were enrolled, the most common clinical severity of MIS-C was moderate. The common presentations of patients were fever (97%), nonpurulent conjunctivitis (89.6%), rashes (81.3%), fatigue (81.3%), strawberry tongue (79.2%), and myalgia (68.8%). The most common laboratory findings were lymphopenia (81.2%), thrombocytopenia (54.1%), elevated D-dimer levels (89.5%), C-reactive protein (CRP; 100%), procalcitonin (97%), erythrocyte sedimentation rate (87.5%), ferritin (95.8%), interleukin 6 (IL-6) (86.1%), and probrain natriuretic peptide (pro-BNP) (97%). High levels of CRP, procalcitonin, pro-BNP, and urea were associated with the severity of MIS-C (p < 0.05). Fifteen of the patients were found to have pulmonary involvement. Ascites were the most common finding on abdominal ultrasonography (11 patients) and were not seen in a mild form of the disease. During the study period, two patients died. Conclusion It is important to make patient-based decisions and apply a stepwise approach in treating patients with MIS-C due to the increased risk of complications and mortality.

Dermatologic manifestations of multisystem inflammatory syndrome in children during the COVID-19 pandemic

Objectivesmulti-system ınflammatory syndrome in children (MIS-C) is an immune-mediated process that develops after infections like SARS-CoV-2. The authors aimed to reveal the mucocutaneous findings of patients diagnosed with MIS-C at presentation and evaluate the frequency of these mucocutaneous findings and their possible relationship with the severity of the disease. MethodsA prospective study was conducted of 43 children admitted to a tertiary hospitals between January 2021 and January 2022 who met Centers for Disease Control and Prevention criteria for MIS-C. Results43 children (25 [58.1%] male); median age, 7.5 years [range 0.5‒15 years]) met the criteria for MIS-C. The most common symptom was cutaneous rash 81.4%, followed by gastrointestinal symptoms 67.4%, oral mucosal changes 65.1%, and conjunctival hyperemia 58.1%. The most common mucosal finding was fissured lips at 27.9%, diffuse hyperemia of the oral mucosa at 18.6%, and strawberry tongue at 13.9%. Urticaria (48.8%) was the most common type of cutaneous rash in the present study’s patients. The most common rash initiation sites were the trunk (32.6%) and the palmoplantar region (20.9%). The presence or absence of mucocutaneous findings was not significantly associated with disease severity. Study limitationsThe number of patients in the this study was small. ConclusionsThe present study’s prospective analysis detected mucocutaneous symptoms in almost 9 out of 10 patients in children diagnosed with MIS-C. Due to the prospective character of the present research, the authors think that the characteristic features of cutaneous and mucosal lesions the authors obtained will contribute to the literature on the diagnosis and prognosis of MIS-C.

68 The confounder in time of confusion: a case report on Multisystem Inflammatory Syndrome in Children (MIS-C)

68 The confounder in time of confusion: a case report on Multisystem Inflammatory Syndrome in Children (MIS-C)

26 Kawasaki’s disease vs systemic juvenile idiopathic arthritis, a diagnostic dilemma

BackgroundBoth these conditions are separate inflammatory conditions whose clinical presentation overlap and thus make their distinction difficult. Each has a criterion that allows diagnosis but since features overlap, diagnoses may be missed.Recent research also shows that both disease processes have similar underlying pathogenesis and involve interleukin 1; which have now raised the question of whether both these disease processes are distinct entities or conditions of a similar spectrum.MethodsMedical records of from the inpatient admission at current and referring hospitals which included clinical history and examination, laboratory and radiological findings.Results13-year-old boy referred from Isiolo county with complains of unremitting fevers for 9 days with associated hip pains. Was initially treated for malaria, and started on broad spectrum antibiotics and antivirals. On further examination, was noted to have cracked lips, strawberry tongue, non-suppurative conjuctivitis and an anterior chest wall wheal like rash. Examination also revealed a left sided non tender submandibular lymph node. Initial laboratory investigations showed neutrophilia of 12.89, thrombocytosis of 431, CRP of 364 and normal joint radiographs. An ECHO done showed left ventricular enlargement with corresponding mild Mitral regurgitation. Blood cultures showed no organisms. Autoimmune markers and tropical fever markers were normal as well. He was then placed on prednisolone challenge and reported to be fever free for the first time in 12 days.Discussion and conclusionA persistent fever unremitting with parenteral antipyretics can have a multitude of differentials. In a tropical nation the likelihood of an infectious cause is more common than other aetiologies. Hence, it should always be investigated for thoroughly. In the case highlighted above, malaria, TB, osteomyelitis, septic arthritis and other infectious causes were investigated. Childhood malignancy can also present with the same. But of note is autoimmune conditions need to be considered. With history of arthritic like pain, quotidian pattern of fevers, evanescent salmon coloured chest wall rash and cervical lymphadenopathy, a diagnosis of Systemic Juvenile Arthritis needs to be considered. Often this may be overlooked or misinterpreted as Kawasaki’s; which is a close differential; or Still’s disease. There is a criterion for SJIA by the International League of Associations for Rheumatology, however, there can be an incomplete presentation as well. This adds on to the diagnostic challenge and hence, SJIA may remain undiagnosed for a long time.

Clinical characteristics and outcomes of the multisystem inflammatory syndrome in children (MIS-C) following COVID-19 infection in Iran: A multicenter study.

ObjectivesThis study aimed to assess the clinical characteristics, treatment and outcomes of the multisystem inflammatory syndrome in children (MIS-C) following COVID-19 in five different geographical regions of Iran.MethodsIn this multicenter observational study, patients <21 years were included between March 2020 and October 2021. By Disease Control and Prevention (CDC) checklist, demographic characteristics, comorbidities, clinical signs and symptoms, laboratory and radiology findings, and treatment were collected. Statistical analysis was using Chi-square and t-test in STATA14.ResultsIn total 225 patients with median age of 55 (26–96) months were included that 59.56% boys. 57.33% were admitted to the PICU with a median of 7 days (4–10). 95.56% of patients were discharged with recovery and the rest died. All of the patients in our study were included based on the MIS-C criteria. However, some patients had Kawasaki symptoms, so we compared the clinical and epidemiological characteristics of the two groups. Conjunctival injection, cervical lymphadenopathy>1.5 cm diameter, and strawberry tongue in Kawasaki-like MIS-C patients were higher than of MIS-C patients, and this difference was significant(p<0.001). The most common comorbidity was obesity (24.86%). Most patients tested for COVID-19 and about 60% of the patients had a positive test by serology or reverse transcription-polymerase chain reaction (RT-PCR). Gastrointestinal (88.89%) and hematologic signs (84.44%) were most common. Most drugs used in patients were IVIG and steroids. 88.07% and 61.29% of the patients had at least one problem in echocardiography and lung CT, respectively.ConclusionsThe best outcome was seen in patients who were treated with both IVIG and steroids on the first days of admission. Myocarditis was common in two groups of patients. According to most patients had echocardiography abnormal, screening of heart function is recommended for patients.

Multisystem Inflammatory Syndrome in Children and Long COVID: The SARS-CoV-2 Viral Superantigen Hypothesis.

Multisystem inflammatory syndrome in children (MIS-C) is a febrile pediatric inflammatory disease that may develop weeks after initial SARS-CoV-2 infection or exposure. MIS-C involves systemic hyperinflammation and multiorgan involvement, including severe cardiovascular, gastrointestinal (GI) and neurological symptoms. Some clinical attributes of MIS-C—such as persistent fever, rashes, conjunctivitis and oral mucosa changes (red fissured lips and strawberry tongue)—overlap with features of Kawasaki disease (KD). In addition, MIS-C shares striking clinical similarities with toxic shock syndrome (TSS), which is triggered by bacterial superantigens (SAgs). The remarkable similarities between MIS-C and TSS prompted a search for SAg-like structures in the SARS-CoV-2 virus and the discovery of a unique SAg-like motif highly similar to a Staphylococcal enterotoxin B (SEB) fragment in the SARS-CoV-2 spike 1 (S1) glycoprotein. Computational studies suggest that the SAg-like motif has a high affinity for binding T-cell receptors (TCRs) and MHC Class II proteins. Immunosequencing of peripheral blood samples from MIS-C patients revealed a profound expansion of TCR β variable gene 11-2 (TRBV11-2), which correlates with MIS-C severity and serum cytokine levels, consistent with a SAg-triggered immune response. Computational sequence analysis of SARS-CoV-2 spike further identified conserved neurotoxin-like motifs which may alter neuronal cell function and contribute to neurological symptoms in COVID-19 and MIS-C patients. Additionally, autoantibodies are detected during MIS-C, which may indicate development of post-SARS-CoV-2 autoreactive and autoimmune responses. Finally, prolonged persistence of SARS-CoV-2 RNA in the gut, increased gut permeability and elevated levels of circulating S1 have been observed in children with MIS-C. Accordingly, we hypothesize that continuous and prolonged exposure to the viral SAg-like and neurotoxin-like motifs in SARS-CoV-2 spike may promote autoimmunity leading to the development of post-acute COVID-19 syndromes, including MIS-C and long COVID, as well as the neurological complications resulting from SARS-CoV-2 infection.

Epidemiological and Clinical Features of Kawasaki Disease During the COVID-19 Pandemic in the United States

Public health measures implemented during the COVID-19 pandemic had widespread effects on population behaviors, transmission of infectious diseases, and exposures to environmental pollutants. This provided an opportunity to study how these factors potentially influenced the incidence of Kawasaki disease (KD), a self-limited pediatric vasculitis of unknown etiology. To examine the change in KD incidence across the United States and evaluate whether public health measures affected the prevalence of KD. This multicenter cohort study included consecutive, unselected patients with KD who were diagnosed between January 1, 2018, and December 31, 2020 (multicenter cohort with 28 pediatric centers), and a detailed analysis of patients with KD who were diagnosed between January 1, 2002, and November 15, 2021 (Rady Children's Hospital San Diego [RCHSD]). For the multicenter cohort, the date of fever onset for each patient with KD was collected. For RCHSD, detailed demographic and clinical data as well as publicly available, anonymized mobile phone data and median household income by census block group were collected. The study hypothesis was that public health measures undertaken during the pandemic would reduce exposure to the airborne trigger(s) of KD and that communities with high shelter-in-place compliance would experience the greatest decrease in KD incidence. A total of 2461 KD cases were included in the multicenter study (2018: 894; 2019: 905; 2020: 646), and 1461 cases (median [IQR] age, 2.8 years [1.4-4.9 years]; 900 [61.6%] males; 220 [15.1%] Asian, 512 [35.0%] Hispanic, and 338 [23.1%] White children) from RCHSD between 2002 and 2021 were also included. The 28.2% decline in KD cases nationally during 2020 (646 cases) compared with 2018 (894 cases) and 2019 (905 cases) was uneven across the United States. For RCHSD, there was a disproportionate decline in KD cases in 2020 to 2021 compared with the mean (SD) number of cases in earlier years for children aged 1 to 5 years (22 vs 44.9 [9.9]; P = .02), male children (21 vs 47.6 [10.0]; P = .01), and Asian children (4 vs 11.8 [4.4]; P = .046). Mobility data did not suggest that shelter-in-place measures were associated with the number of KD cases. Clinical features including strawberry tongue, enlarged cervical lymph node, and subacute periungual desquamation were decreased during 2020 compared with the baseline period (strawberry tongue: 39% vs 63%; P = .04; enlarged lymph node: 21% vs 32%; P = .09; periungual desquamation: 47% vs 58%; P = .16). School closures, masking mandates, decreased ambient pollution, and decreased circulation of respiratory viruses all overlapped to different extents with the period of decreased KD cases. KD in San Diego rebounded in the spring of 2021, coincident with lifting of mask mandates. In this study of epidemiological and clinical features of KD during the COVID-19 pandemic in the United States, KD cases fell and remained low during the period of masking and school closure. Mobility data indicated that differential intensity of sheltering in place was not associated with KD incidence. These findings suggest that social behavior is associated with exposure to the agent(s) that trigger KD and are consistent with a respiratory portal of entry for the agent(s).

A case report of scarlet fever: An uncommon diagnosis for fever and rash

Streptococcus pyogenes is a pathogen capable of causing a wide spectrum of diseases including pharyngitis and streptococcal toxic shock syndrome, with a range of complications and outcomes. Even though it is not an uncommon pathogen, reports of scarlet fever are scarce in Sri Lanka. We describe a case of scarlet fever in a seven-year-old boy with typical features. The child presented with high fever, body aches, vomiting, loss of appetite and a rash and developed pharyngitis and a strawberry tongue during the ward stay. A pure growth of group A Streptococcus was isolated from a throat swab taken on admission. He improved with appropriate treatment and recovered without complications. The importance of throat swab culture and clinical correlation is illustrated in this case report.

Childhood-Onset Systemic Lupus Erythematosus Presenting with Features Mimicking Kawasaki Disease

Childhood-onset systemic lupus erythematosus (cSLE) may rarely present initially with features mimicking Kawasaki disease (KD). Here, we report a 10-year-old girl presenting with high-grade fever, generalized skin rash, conjunctivitis, cervical lymphadenopathy, dry fissured lips, strawberry tongue, polyarthritis, abdominal pain, and neck stiffness. She was initially diagnosed and treated as KD, and the diagnosis of cSLE was confirmed 4 weeks later when she presented with a malar rash, nasal ulcer, seizures, and polyarthritis. In addition, her antinuclear antibodies, anti-double-stranded DNA, and direct Coombs test were positive. Therefore, with the difficulties in differential diagnosis and the rarity of such presentation, children with KD who develop new or unexpected features need to be evaluated for the development of cSLE.