Bilateral Non-purulent Conjunctivitis Research Articles

The Clinical and Molecular Findings of Patients with Multisystem Inflammatory Syndrome in Children (MIS-C)

Background: several children with COVID-19 disease present with fever, gastrointestinal symptoms, rash, conjunctivitis, mucosal changes, shock, and myocardial dysfunction, called multisystem inflammatory syndrome in children (MIS-C), similar to incomplete Kawasaki disease or toxic shock syndrome. The unclear pathophysiology of MIS-C prompts clinical and genetic evaluation of the patients.
 Method: In the present study, patients with MIS-C disease who were referred by medical specialists of Imam Ali Hospital of Alborz in 2020-2021 were included. The clinical manifestations and laboratory findings of enrolled patients were evaluated, and the genetic analysis was performed by whole exome sequencing (WES), further confirmed by Sanger sequencing.
 Results: Among 11 patients diagnosed with MISC, six patients (54.5%) were male, and the mean (SD) age of participants was 6.55 (±2.81) years. The most prevalent clinical presentations included fever (100%), rash (82%), bilateral non-purulent conjunctivitis (73%), and mucous membrane changes (64%). The only patient with genetic alterations was an 8-year-old boy with a homozygous missense variant of the ATP6AP1 gene and a heterozygous likely pathogenic canonical splice site variant of the M1B1 gene.
 Conclusion: Although the young age of patients with MIS-C and their autoinflammatory presentations are similar to patients with inborn errors of immunity, the results indicate that most patients with MIS-C do not have genetic mutations.

Kawasaki disease in an infant: a case report

Background: Kawasaki disease is an acute systemic vasculitis of childhood, with a predilection of the main coronary artery. This disease is the most frequent cause of acquired heart disease in developed countries. This report is aimed to describe the diagnosis and treatment of Kawasaki disease in an infant. Case description: A 4-month-old boy came to the emergency room on November 21, 2021, with a chief complaint of high fever for three days. His appetite has decreased since he got sick. Other complaints, such as red eyes, red lips and red rashes overall his body, occurred two days before the admission. Physical examination: the child's nutritional status was good, with bilateral non-purulent conjunctivitis, erythema and cracked lips, and polymorphous rash on the body, back, and legs. We found enlarged cervical lymph nodes and reactivation of Bacillus Calmette-Guerin (BCG) scars. The laboratory findings were as follows: HGB 10.6gr/dL, WBC 11.570/µL, PLT 335.000/µL, CRP 84mg/dL, Antigen NS-1 dengue test negative, and SARS-COV2 Antigen test negative. The echocardiography results were minimal pericardial effusion and normal coronary size. With a Harada score of 4, he was diagnosed with Kawasaki disease. Management of the patient includes breastfeeding, infusion of dextrose 5% with sodium chloride (Na Cl) 0.22%, Paracetamol infusion, Intravenous Immunoglobulin (IVIg) single dose, and Acetylsalicylic acid orally. After being discharged from the hospital, he was advised to control his condition routinely to a pediatrician. Conclusion: Kawasaki disease has been reported in a 4-month-old boy with typical clinical manifestations and successfully treated with good results.

"Multisystem Inflammatory Syndrome in Children" (MIS-C) after COVID-19 Infection in the Metropolitan Area of Nuremberg-Erlangen, Germany-Expectations and Results of a Two-Year Period.

Multisystemic Inflammatory Syndrome in children (MIS-C) is a rare autoimmune disorder occurring after a latency period following acute SARS-CoV-2 infection. The therapeutic regime of MIS-C is adapted to the therapy of the Kawasaki disease, as clinical symptoms are similar. Since the Kawasaki disease can potentially result in severe symptoms, which may even affect long-term health, it is essential to gain further knowledge about MIS-C. Thus, we aimed to investigate the incidence, symptoms, therapeutical procedure and outcome of MIS-C patients in the metropolitan area of Nuremberg-Erlangen during the SARS-CoV2 pandemic. Retrospective analysis of clinical charts of MIS-C patients was carried out at three children's hospitals covering the medical care of the metropolitan area of Nuremberg-Erlangen in Germany. Demographic characteristics and symptoms at first visit, their clinical course, therapeutic regime and outcome were recorded within the time period January 2021-December 2022. Analysis of 10 patients (5 male, 5 female) with MIS-C resulting in an incidence of 2.14/100.000 children. The median time between COVID-19 infection and admission to hospital was 5 weeks. The median age was 7 years. Symptoms comprised fever (100%), rash (70%), bilateral non-purulent conjunctivitis (70%) and urticaria (20%). At the time of presentation, diagnosis-defining inflammation parameters were increased and the range for C-reactive protein was 4.13 mg/dL to 28 mg/dL, with a median of 24.7 mg/dL. Procalcitonin was initially determined in six patients (1.92 ng/mL to 21.5 ng/mL) with a median value of 5.5 pg/mL. Two patients displayed leukocytosis and two displayed leukopenia. None of the patients presented coronary pathologies. Nine of the ten patients received intravenous immunoglobulin (IVIG) therapy. In addition, patients received intravenous steroids (80%) and acetylsalicylic acid (80%). SARS-CoV virus may rarely exert multiorgan manifestations due to hyperinflammatory immunological processes. Within two years of the COVID-19 pandemic, we identified ten patients with COVID-induced MIS-C in the metropolitan area Nuremberg-Erlangen. In the description of the patient collective, we can confirm that MIS-C is distinguished from the Kawasaki disease by the lack of coronary manifestations. Interestingly, although having monitored all pediatric facilities in the investigated area, we find lower incidences of MIS-C compared to findings in the literature. In conclusion, an overestimation of incidences in the upcoming MIS-C during the pandemic needs to be considered.

Severe Dengue or Covid-19 Related MIS-C? Diagnostic Challenges in Resource-Limited and Dengue-Endemic Regions

Besides serological cross-reactivity and co-infection between dengue and SARS-CoV-2, diagnostic challenges arise because many people have had positive dengue IgG in dengue-endemic regions, and patients usually come after 4-5 days of fever. We report a case of 9-year-old girl, diagnosed with dengue with warning signs on admission because of fever, severe abdominal pain, thrombocytopenia, and positive dengue IgG. The subsequent course of the illness was not consistent with dengue infection because the shock happened during the febrile phase concurrent with rising leucocyte and platelet counts, the fever continued for longer than seven days, and there was bilateral non-purulent conjunctivitis with subconjunctival hemorrhage. She was diagnosed with MIS-C because of positive SARS-CoV-2 IgG and hyperinflammatory markers. Her clinical condition improved progressively after steroid administration. Clinical awareness about MIS-C is required to avoid misdiagnosis and improper treatment during or after the COVID-19 pandemic. Every clinician should consider MIS-C as a differential diagnosis if patients have inconsistencies with the course of dengue illness.

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

Cutaneous manifestations of paediatric multisystem inflammatory syndrome temporally associated with SARS ‐ CoV ‐2: a single‐centre experience

Paediatric inflammatory multisystem syndrome associated with severe acute respiratory syndrome–coronavirus 2 (SARS-CoV-2) infection (PIMS-TS) is defined as ‘a child presenting with persistent fever, inflammation and evidence of single or multi-organ dysfunction with additional features which include a rash’.1 The World Health Organisation’s (WHO) definition further includes a ‘rash or bilateral non-purulent conjunctivitis or muco-cutaneous inflammation signs’.2 Previously reported mucocutaneous manifestations (MCM) in PIMS-TS have been vague. This study aimed to delineate the MCM of children presenting with PIMS-TS in more detail.

A Rare Case of Kawasaki-like Multisystem Inflammatory Syndrome following COVID-19 in an Adult

In this case report, we describe a Kawasaki-like multisystem inflammatory syndrome (MIS-A) in a 33-year old man that occurred 19 days after a SARS-CoV-2 infection. The main features at presentation were profound myocarditis, bilateral non-purulent conjunctivitis, mediastinal lymphadenopathy, and acute kidney failure and laboratory evidence of hyperinflammation. He received ACE-inhibition and beta-blockers for his heart failure and made a fairly rapid spontaneous recovery over the subsequent 8 days.

Acute severe necrotizing pancreatitis: A manifestation of multisystem inflammatory syndrome in children?

Background: Multisystem inflammatory syndrome in children (MIS-C) is commonly being diagnosed among children, 2–8 weeks following a severe acute respiratory syndrome (SARS)-CoV-2 infection. Several cases of pancreatitis have been reported with SARS-CoV-2 infection in adults but only one in a 10-year-old girl with MIS-C. Clinical Description: During the coronavirus disease (COVID) pandemic, a 1-year-old girl presented with high-grade fever for 3 days and vomiting and abdominal pain for a day. Her parents had contracted SARS COVID-2 infection 5 weeks earlier. At admission, she was febrile, drowsy, had tachycardia, tachypnea, and hypotension. Salient examination findings included bilateral nonpurulent conjunctivitis, diminished air entry and crepitation's in the left basal zone, distended abdomen with guarding and tenderness in the left hypochondrium and epigastrium. The diagnostic criteria of MIS-C were fulfilled, but not for classical or incomplete Kawasaki disease. Biochemical markers and radiological findings confirmed acute severe necrotizing pancreatitis. No other etiological cause of pancreatitis could be identified. Management: Intravenous immunoglobulins were started as per protocol. Steroids were withheld in view of the pancreatitis. The child showed dramatic resolution in fever and rapid improvement in clinical and biochemical parameters. Conclusion: Pancreatitis may be a presentation of MIS-C, either due to a direct cytopathic effect or secondary to a hyper-inflammatory response. A high index of suspicion should be kept in children with fever and severe pain abdomen with recent history of COVID-19 infection in the patient or close contacts.

Pediatric inflammatory multisystem syndrome temporally associated with COVID-19: a spectrum of diseases with many names

KEY POINTS A 10-year-old previously healthy boy presented to the emergency department with 6 days of persistent fever; 4 days of abdominal pain, emesis and diarrhea; and bilateral nonpurulent conjunctivitis and red cracked lips. Four weeks before presentation, the patient had had 3 days of headache

2415 Seeing Is Believing: Conjunctival Suffusion in a Jaundiced Patient Diagnosed With Leptospirosis

INTRODUCTION: The broad differential in the workup of infectious causes of abnormal liver enzymes can be narrowed by a good history and physical. Bilateral Conjunctival suffusion, characterized by non-purulent conjunctivitis, is present in 55% of patients with leptospirosis and is uncommon in other infectious diseases. We report a case of leptospirosis in a patient with conjunctival suffusion, abnormal liver enzymes, jaundice, and recurrent fevers. CASE DESCRIPTION/METHODS: A 44-year-old previously healthy Hispanic male presented with painless jaundice, subjective fevers, nonproductive cough, and a morbilliform rash for 14 days. He reported no recent travel, sick contacts, bad habits, or hepatotoxic herbal medications, and he worked in a slaughterhouse. He denied joint pain, abdominal pain, and pruritus. On exam, he had a temperature of 38.2°C and was tachycardic to 117 with normal blood pressure. He had bilateral non-purulent conjunctivitis with mild scleral icterus and a faint morbilliform rash over both his thighs. Laboratory data was remarkable for leukocytosis (14.9 WBC/mm3), elevated bilirubin (4 mg/dL total, 2.3 mg/dL direct), Alkaline phosphatase (517 U/L), AST (84 U/L) and ALT (162 U/L). Imaging studies revealed hepatic steatosis with no other findings. Infectious workup indicated that leptospira antibody to IgM were likely present based on DOT BLOT testing (ARUP labs). Serologic testing for other infectious causes were unremarkable. The patient was started on doxycycline 100 mg twice a day for one week for mild leptospirosis with improvement in his symptoms, labs, and clinical exam. DISCUSSION: Leptospirosis, although endemic in the tropics, is a rare cause of jaundice and abnormal liver enzymes in the United States. It is associated with environmental exposure such as animal urine, contaminated water or soil, or infected animal tissue. Gastrointestinal clinical features include abdominal pain, jaundice, elevated hepatic transaminases and serum bilirubin. An important but frequently overlooked clinical finding is bilateral conjunctival suffusion, which is rare in other infectious diseases. Diagnosis is made by serologic testing for IgM antibodies. Treatment involves supportive care with oral doxycycline in mild cases and IV antibiotics for hospitalized patients. An astute physician should have a high index of suspicion for leptospirosis if non-purulent conjunctivitis is found in the infectious workup of fever and abnormal liver enzymes as it may save time and prevent excessive testing.

Kawasaki Disease in the neonate: case report and literature review

BackgroundKawasaki Disease (KD), the leading cause of acquired heart disease in children in the developed world, is extremely rare in neonates. We present a case of incomplete KD in a neonate and a review of the literature on neonatal KD.Case presentationA previously healthy full term 15 day old Caucasian male with an unremarkable antenatal and perinatal history, presented on Day 2 of illness with fever, rash, irritability, and poor feeding. Examination revealed fever (39.6C), tachycardia, tachypnea, extreme irritability, and a generalized maculopapular rash, but was otherwise normal. His complete blood count, CRP and ESR were normal. Empiric intravenous antibiotics and acyclovir resulted in no improvement. On day 4, he had ongoing fever and developed recurrent apnea, required supplemental oxygen, and was transferred to the pediatric intensive care unit. On day 6, he developed bilateral non-purulent conjunctivitis, palmar erythema, bilateral non-pitting edema and erythema of his feet, and arthritis. His full septic work-up and viral studies were negative. On Day 7 he was treated with intravenous immunoglobulin, and over the next 48 h his symptoms including extremity edema resolved, he no longer required supplemental oxygen, and fever did not recur. On day 9 of illness he had marked thrombocytosis. Subsequently, he developed distal extremity desquamation. Repeated echocardiograms excluded the presence of coronary artery aneurysms (CAA).ConclusionsWe believe this to be a rare case of incomplete KD in a neonate, in which timely IVIG administration led to resolution of the acute illness and may have prevented CAA. A comprehensive English-language medical literature review of KD presenting in the neonatal period revealed only fifteen case reports. Cases often presented with incomplete KD, and a number had atypical laboratory features including a normal CRP in the acute phase, similar to what was seen in our patient. This case and our literature review should increase awareness that KD can rarely occur in neonates, often presenting atypically. Recognizing KD in a neonate enables appropriate treatment that can result in resolution of symptoms and may decrease the risk of cardiac complications.

The Clinical Diagnosis and Management of Kawasaki Disease: a Review and Update.

Kawasaki disease is an acute, self-limited vasculitis of childhood and has become the leading cause of acquired pediatric heart disease in the USA. Prompt treatment is essential in reducing cardiac-related morbidity and mortality. The underlying etiology remains unknown. The disease itself may be the characteristic manifestation of a common pathway of immune-mediated vascular inflammation in susceptible hosts. The characteristic clinical features of fever for at least 5 days with bilateral nonpurulent conjunctivitis, rash, changes in lips and oral cavity, changes in peripheral extremities, and cervical lymphadenopathy remain the mainstay of diagnosis. Supplementary laboratory criteria can aid in the diagnosis, particularly in cases of incomplete clinical presentation. Diagnosis of Kawasaki disease can be challenging as the clinical presentation can be mistaken for a variety of other pediatric illnesses. Standard of care consists of intravenous immune globulin and aspirin. Corticosteroids, infliximab, and cyclosporine A have been used as adjunct therapy for Kawasaki disease refractory to initial treatment. There is ongoing research into the use of these agents in the initial therapy of Kawasaki disease.

Bilateral optic neuritis in a patient with Kawasaki disease

We describe a 7-year-old male with a history of fever, headache, bilateral non-purulent conjunctivitis, and photophobia. Patient was initially treated for suspected bacterial meningitis with intravenous antibiotics. Due to lack of clinical response, negative cultures, and evolving signs of Kawasaki disease, he was treated with one dose of intravenous immunoglobulin. Patient improved except for the persistence of photophobia and bilateral conjunctival injection. Eye examination showed bilateral optic neuritis and a visual acuity of 20/80 in both eyes. Follow up eye examination after 6 weeks showed normal visual acuity, resolution of conjuctival injection, photophobia, and optic neuritis. This case highlights the posterior segment involvement in Kawasaki disease and the need for careful eye examination in patients with Kawasaki disease who have prolonged photophobia and conjunctival injection.

G284 Kawasaki disease bpsu survey in great britain and ireland

Introduction Kawasaki disease (KD) is the commonest cause of acquired heart disease in the western world. We report here on the preliminary data for the first year of a 2-year national survey in Great Britain and Northern Ireland. Methods Using standard BPSU methodology, children with Complete KD (for the first year) presenting from January 1st to December 31st 2013 were studied with their treatments and complications. The steering committee reviewed cases that were unclear or incomplete. Results Excluding incomplete KD and incomplete data sets, there were 180 with complete KD (15/month). Peak presentation was in March, boy:girl ratio was 1.8 and 69% were 1 to 4 years old. The highest incidence was in children under 5 years especially in London and East Anglia (5.6 / 100,000) and lowest in the Midlands (2.8/100,000). Highest incidence was in Chinese (21.9/100,000), Black African and Caribbean (14.5/100,000) and Mixed ethnicity (15.3/100,000). 81% saw a GP at median 2 days after fever commencement and admission was median (range) of 2 (0–27) days later. Fever duration was 6 (1–28) days. 97% had rash, usually at the start of fever, which was on the trunk or widespread in 89% and was macular in 75%. Bilateral non-purulent conjunctivitis (91%) occurred at 4 (0–27) days and mucositis (97%) at 5 (0–27) days. Periphery involvement was up to 32 days in 156 (86%). Lymphadenopathy was least common (65%) often after 5 days. The BCG scar was inflamed in 11. CRP ranged from 1–501 mg/l, albumin from 17–45g/l, lowest platelet counts were below 150 × 10^9/l in 14. 31 (22%) had dilated coronary arteries, 9 had pericardial effusion and 12 had ECG abnormalities. Intravenous immunoglobulin (2g/kg) given within 7 days to 95%, but not to 9 complete cases (delayed diagnosis). Second dose was given to 13 children. High dose aspirin (30–50mg/kg) followed by low dose aspirin was given to 92%. 19 had sequelae, 15 with persistence of coronary artery dilation, with no deaths. Conclusions These preliminary results show a high incidence of coronary artery disease with the old guideline for KD and give new data on seasonality, ethnicity and correlation with clinical symptoms.

Kawasaki Disease Shock Syndrome

Kawasaki disease (KD) is an acute multisystem vasculitis that occurs principally in children younger than 5 years of age. The clinical characteristics of KD include diffuse mucosal inflammation, bilateral nonpurulent conjunctivitis, erythematous skin rashes, angioedema over the hands and feet, and cervical lymphadenopathy in addition to prolonged and unexplained fever lasting for >5 days.Kawasaki disease shock syndrome (KDSS) is rare and severe form of KD. Pediatricians and intensive care unit professionals should be aware of this uncommon presentation of KD to prevent complications. Here in we presented a 2-years old male patient with Kawasaki shock syndrome (KSS)

The clinical profile of Kawasaki disease of children from three Polish centers: a retrospective study

Kawasaki disease (KD) is one of the most common vasculitides of childhood. The aim of this retrospective study is to determine the incidence of KD and to evaluate its presenting symptoms, clinical course, laboratory tests, and treatment in patients with complete KD and incomplete KD at three pediatric rheumatology centers in Poland from January 2011 to December 2012. A total of 27 Caucasian children (12 boys and 15 girls) with median age of 3 years (range 4 months–12 years) were included in this study. The incidence of complete versus incomplete KD was 17 (63 %) versus 10 (37 %) children, respectively. Patients with incomplete KD significantly less presented cervical lymphadenopathy (20 vs. 88.2 %; p = 0.00075), changes in extremities (30 vs. 76.5 %; p = 0.04), and bilateral nonpurulent conjunctivitis (60 vs. 100 %; p = 0.01). Cardiac assessments show that the majority of patients with KD have not got coronary artery aneurysms (CAA). The median time from the onset of symptoms to intravenous immunoglobulin (IVIG) infusion was 7 days for complete KD and 11 days for incomplete KD. IVIG delay in the incomplete KD had no effect on the incidence of CAA. In conclusion, there were no differences in demographic features, age of onset, and laboratory tests of patients with complete and incomplete KD. Patients with incomplete KD significantly rarely presented cervical lymphadenopathy, changes in extremities, and conjunctival injection. Electrocardiography is a sensitive test to recognize cardiac involvement in the acute phase of KD. Despite the fact that incomplete forms of presentation often delay diagnosis, in most patients treatment with IVIG can avoid complication of CAA.

SAT0454 The Retrospective Analysis of Patients with Kawasaki Disease from Three Pediatric Rheumatology Centers in Poland

BackgroundKawasaki Disease (KD) is one of the most common vasculitides of childhood. The diagnosis of Complete KD (C-KD) requires the presence of fever lasting at least 5 days without any...

Incomplete Kawasaki Disease: A Diagnostic Dilemma

Copyright © 2013, Iranian Society of Pediatrics. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/3.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. We read with great interest the recent report by Halimiasl et al. describing an interesting case of a child with a prolonged fever accompanied with coronary artery aneurysm and myocarditis published in your respected journal (1). This study has novelties and enhances the existing literature; however, the diagnostic approach of the authors may be questioned. At the present time, there are two validated and generally accepted criteria for the diagnosis of Kawasaki disease (KD). This includes the 2004 American Heart Association (AHA) criteria (2), and the 2008 Japanese Circulation Society Joint Working Groups (JCS) criteria (3). In AHA criteria, prolonged fever lasting longer than 5 days together with at least four of the five major clinical characteristics of including diffuse mucosal inflammation, bilateral nonpurulent conjunctivitis, dysmorphic skin rashes, indurative angioedema over the hands and feet, and cervical lymphadenopathy are required for the diagnosis of (2). On the other hand, In JCS criteria, prolonged fever is considered as the sixth major clinical presentation, and five of the six mentioned major criteria are required for diagnosis (3). Patients who do not fully meet the criteria for should undergo echocardiogram study for detection of coronary artery abnormalities (CAA). The definitions of CAA in such cases are: maximum absolute internal diameter >3 mm in children lower than 5 years of age or > 4 mm in children 5 years and above, or a segmental diameter 1.5 times greater than that of an adjacent segment or the presence of luminal irregularity. These cases are considered as incomplete or atypical Kawasaki (2). In the report by Halimiasl et al. no sufficient data about the major clinical characteristics of in their patient was provided. Out of six major criteria for KD, only prolonged fever was described in the paper. It is reported that the index case had coronary aneurysm and myocarditis in echocardiogram study. Nevertheless, the question is how the diagnosis of incomplete KD could be made based on only a prolonged fever that was accompanied with cardiac abnormalities but no other major criteria for KD. We were wondering if the authors could provide more data about the clinical/paraclinical characteristics of their patient for the readership, and discuss their diagnostic approach to in this patient in more details.

A 13-year-old adolescent with Kawasaki disease presenting with massive unilateral cervical lymphadenopathy and polyarthritis

A 13-year-old male adolescent suddenly developed unilateral cervical lymphadenopathy which resulted in the excision of the enlarged lymph nodes. After the intervention his general state deteriorated and the patient was transferred to our hospital. The patient presented with high fever, bilateral non-purulent conjunctivitis, strawberry tongue and fissured lips, a maculopapular rash and severe polyarthritis including all peripheral joints. We treated him for Kawasaki disease with single high-dose intravenous gammaglobulin. Within 16 hours he became afebrile and all other symptoms disappeared rapidly. A few days later, he developed thrombocytosis and desquamation of the tips of fingers and toes. The immunohistological examination of the lymph nodes demonstrated interfollicular hyperplasia of the T-zones and in the peripheral blood an expansion of the Vβ2-positive T-cell subset was found. These findings support recent aspects concerning the pathogenesis of Kawasaki disease. Discussion: Although Kawasaki disease is mainly observed in preschoolchildren, older children and adolescents may be affected. Extreme forms of cervical lymphadenopathy may occur and in the case of a strictly unilateral lymphadenopathy the diagnosis may be difficult.