We report the first case demonstrating an association between Kawasaki disease (KD) and erythema nodosum (EN). A 3-year-old girl presented with EN as an initial manifestation of KD. At the initial visit, she showed high fever of 40℃, injection of the oropharynx, cervical lymphadenopathy, and red-purple cutaneous nodules, particularly on the lower limbs. She complained of severe pain in the neck and cutaneous lesions. Initially, the development of EN was attributed to Salmonella spp infection, which was detected in stool culture. However, the patient did not respond to high-dose ampicillin/sulbactam to which the Salmonella spp is sensitive. Echocardiography performed as screening for fever of unknown origin revealed medium-sized aneurysms of the left anterior descending artery. EN masked the diagnosis of KD, and the patient developed a coronary artery lesion. KD should be considered in the differential diagnosis of refractory EN in pediatric patients.
Aneurysm ; Arteries ; Bacterial Infections ; Child, Preschool ; Coronary Vessels ; Diagnosis ; Diagnosis, Differential ; Echocardiography ; Erythema Nodosum ; Erythema ; Exanthema ; Female ; Fever ; Fever of Unknown Origin ; Humans ; Lower Extremity ; Lymphatic Diseases ; Masks ; Mass Screening ; Mucocutaneous Lymph Node Syndrome ; Neck ; Oropharynx ; Salmonella ; Skin ; Subcutaneous Tissue

Sinus histiocytosis with massive lymphadenopathy, also known as Rosai-Dorfman disease (RDD), is a rare histiocytic disorder of unknown etiology. Most patients with RDD have spontaneous remission, but in some patients, the disease recurs after complete remission and may not respond to general treatment. Some patients with RDD involving the extranodal system can have serious symptoms such as vital organ dysfunction due to mass effects, neurological symptoms caused by intracranial involvement, and respiratory distress with airway involvement. We report the case of a 7-year-old girl with severe dyspnea due to refractory extranodal RDD that caused progressive upper airway obstruction. She was admitted because of nasal congestion and persistent cervical lymphadenopathy, and diagnosed as having RDD by cervical lymph node incisional biopsy. The initial prednisone treatment did not improve her symptoms. The following contrast-enhanced neck computed tomography revealed a newly developed airway mass protruding in the upper trachea. After 8 weeks of chemotherapy with vinblastine, methotrexate, and prednisone, complete remission was attained. Seven months after chemotherapy cessation, the disease recurred, and chemotherapy with vincristine, cytarabine, and prednisone was resumed. Despite the chemotherapy and emergency radiotherapy, no improvement was observed in the cervical lymph node enlargement and airway obstructive symptom due to the upper tracheal mass. 2-Chlorodeoxyadenosine (cladribine) therapy was initiated, and the patient got complete remission after 6 cycles of the cladribine treatment and maintained no evidence of disease for 2 years. We suggest that cladribine is an effective treatment option for recurrent/refractory RDD.
Airway Obstruction ; Biopsy ; Child ; Cladribine ; Cytarabine ; Drug Therapy ; Dyspnea ; Emergencies ; Estrogens, Conjugated (USP) ; Female ; Histiocytosis, Sinus ; Humans ; Lymph Nodes ; Lymphatic Diseases ; Methotrexate ; Neck ; Prednisone ; Radiotherapy ; Remission, Spontaneous ; Trachea ; Vinblastine ; Vincristine

Drug reaction with eosinophilia and systemic symptoms(DRESS), which occurs 2–8 weeks after taking a medication is a rare and potentially life-threatening drug-induced hypersensitivity reaction, which includes skin eruption, hematologic abnormalities, lymphadenopathy, and internal organ such as liver, lung, kidney involvement. Antiepileptic agents (e.g., carbamazepine, lamotrigine, phenytoin, and phenobarbital) and allopurinol are the most commonly reported causes. However, new antiepileptic agents, such as oxcarbazepine, rarely cause drug reaction with eosinophilia and systemic symptoms. A 11-year-old boy who was administered oxcarbazepine for 34 days developed widespread rashes, facial edema, fever, cough, nasal stuffiness, tonsillitis, and cervical lymphadenopathy. Laboratory test results showed leukocytosis, eosinophilia, thrombocytosis, elevated c-reactive protein, and elevated liver transaminase levels. As we suspected drug reaction with eosinophilia and systemic symptoms, we immediately withdrew oxcarbazepine and commenced corticosteroid therapy. The patient's skin lesions and abnormal laboratory results slowly improved. Before change the antiepileptic agents, we performed human leukocyte antigen (HLA) typing to assess the genetic risk factors of the drug reaction and the result was positive for HLA DRB1*04:03 known to cause severe acute drug hypersensitivity, such as Stevens-Johnson syndrome by oxcarbazepine in Koreans. We have presented the first report of drug reaction with eosinophilia and systemic symptoms associated with oxcarbazepine in a patient with HLA DRB1*04:03. Although DRESS by oxcarazepine is extremely rare and unpredictable, when suspected clinical symptoms occur, it is necessary to interrupt the causative drug rapidly and confirming the patient's HLA typing may help to select a safer alternative drug.
Allopurinol ; Anticonvulsants ; C-Reactive Protein ; Carbamazepine ; Child ; Cough ; Drug Eruptions ; Drug Hypersensitivity ; Drug Hypersensitivity Syndrome* ; Edema ; Eosinophilia ; Exanthema ; Fever ; Histocompatibility Testing ; Humans ; Hypersensitivity ; Kidney ; Leukocytes ; Leukocytosis ; Liver ; Lung ; Lymphatic Diseases ; Male ; Palatine Tonsil ; Phenytoin ; Risk Factors ; Skin ; Stevens-Johnson Syndrome ; Thrombocytosis ; Tonsillitis

PURPOSE: The purpose of this study was to investigate the clinical significance of Bacille Calmette-Guérin (BCG) site reaction in terms of diagnosis and outcome prediction in young children with Kawasaki disease (KD). METHODS: The incidence of BCG site reaction in the respective age ranges was investigated in 1,058 patients who were admitted at Asan Medical Center between January 2006 and February 2017. The 416 patients under 18 months of age were enrolled as subjects for the analysis of the association between BCG site reaction and other laboratory and clinical findings. The analysis was performed separately in complete and incomplete KD groups. RESULTS: The incidence rate of BCG site reaction was peaked at 6–12 months (83%) and decreased with increasing age after 12 months in 1,058 patients (P < 0.001). The incidence rate was above 70% in KD aged less than 18 months and more frequent than those of cervical lymphadenopathy. The logistic regression analyses showed that the principal clinical findings including conjunctivitis (P=0.781), red lips/oral mucosa (P=0.963), rash (P=0.510), cervical lymphadenopathy (P=0.363), changes in extremities (P=0.283) and the coronary artery aneurysm (P=0.776) were not associated with the BCG site reaction. CONCLUSIONS: The BCG site reaction could be a useful diagnostic tool independent to principal clinical findings in KD developing in children aged < 18 months, who underwent BCG vaccination. Outcome of KD patients was not different between groups with or without the BCG site reaction in both complete KD and incomplete KD.
Aneurysm ; BCG Vaccine ; Child ; Chungcheongnam-do ; Conjunctivitis ; Coronary Vessels ; Diagnosis ; Erythema ; Exanthema ; Extremities ; Humans ; Incidence ; Logistic Models ; Lymphatic Diseases ; Mucocutaneous Lymph Node Syndrome ; Mucous Membrane ; Mycobacterium bovis ; Vaccination

Epstein-Barr virus (EBV) infection can be presented with various clinical manifestations and different levels of severity when infected. Infectious mononucleosis, which is most commonly caused by EBV infection in children and adolescents, is a clinical syndrome characterized by fatigue, malaise, fever, sore throat, and generalized lymphadenopathy. But rarely, patients with infectious mononucleosis may present with gastrointestinal symptoms and complicated by gastritis, splenic infarction, and splenic rupture. We encountered a 16-year-old girl who presented with fever, fatigue, and epigastric pain. Splenic infarction and EBV-associated gastritis were diagnosed by using esophagogastroduodenoscopy and abdominal computed tomography. Endoscopy revealed a generalized hyperemic nodular lesion in the stomach, and the biopsy findings were chronic gastritis with erosion and positive in situ hybridization for EBV. As splenic infarction and acute gastritis are rare in infectious mononucleosis and are prone to be overlooked, we must consider these complications when an infectious mononucleosis patient presents with gastrointestinal symptom.
Adolescent ; Biopsy ; Child ; Endoscopy ; Endoscopy, Digestive System ; Epstein-Barr Virus Infections ; Fatigue ; Female ; Fever ; Gastritis* ; Herpesvirus 4, Human* ; Humans ; In Situ Hybridization ; Infectious Mononucleosis ; Lymphatic Diseases ; Pharyngitis ; Splenic Infarction* ; Splenic Rupture ; Stomach

Kikuchi-Fujimoto disease (KFD) is a benign and self-limited disease characterized by fever and lymphadenopathy. The etiology of KFD is unknown, but an autoimmune cause has been suggested. Hashimoto thyroiditis is the most common autoimmune thyroid disorder in children and is known to be associated with other autoimmune diseases. Only a few cases of Hashimoto thyroiditis associated with KFD have been documented. We report a case of a 16-year-old girl who was first diagnosed with KFD and developed Hashimoto thyroiditis 2 years and 6 months later during her follow-up period. Physicians of patients with KFD should consider the possibility of autoimmune diseases like Hashimoto’s thyroiditis.
Adolescent ; Autoimmune Diseases ; Child ; Female ; Fever ; Follow-Up Studies ; Hashimoto Disease* ; Histiocytic Necrotizing Lymphadenitis* ; Humans ; Lymphatic Diseases ; Thyroid Gland ; Thyroiditis

OBJECTIVES: Kikuchi-Fujimoto disease (KFD) is characterized by lymphadenopathy and fever, and is usually self-limited. This study analyzed the clinical characteristics of pediatric patients with KFD. METHODS: This retrospective, observational, single-center study was conducted in South Korea from March 2008 to October 2015. KFD was diagnosed based on clinical, radiological or histological findings and excluded when there were any other causes of lymphadenopathy. Medical records were reviewed for clinical and laboratory manifestations. RESULTS: A total of 35 cases were included. The mean patient age was 12.1±2.9 years (range, 5 to 17 years); the male-to-female ratio was 1:0.8. The main clinical manifestations were cervical lymphadenopathy and fever in 34 cases (97%). The mean duration of fever was 12.2±8.3 days (range, 2 to 37 days). We noted enlargement of lymph nodes in the cervical, mesenteric (n=5, 14%), axillary (n=2, 6%), and inguinal (n=1, 3%) regions. Hepatosplenomegaly, loss of appetite, and rash were observed. On laboratory examinations, elevation of ferritin, leukopenia, and positivity for anti-nuclear antibodies were frequently observed. Twelve patients underwent biopsy and 23 cases were diagnosed by radiological findings. The mean duration of hospitalization for all cases was 7.9±2.9 days (range, 3 to 13 days) and steroids were administered in 10 cases. KFD recurrence was observed in 2 cases (5.7%) with the time to relapse of 7 months and 4 years. There were no cases with systemic lupus erythematous or other autoimmune disease. CONCLUSION: KFD should be considered in pediatric patients with lymphadenopathy and prolonged fever. Patients with KFD should be monitored for recurrence and the development of autoimmune disease.
Adolescent* ; Antibodies ; Appetite ; Autoimmune Diseases ; Biopsy ; Child* ; Exanthema ; Ferritins ; Fever ; Histiocytic Necrotizing Lymphadenitis* ; Hospitalization ; Humans ; Korea ; Leukopenia ; Lymph Nodes ; Lymphatic Diseases ; Medical Records ; Pediatrics ; Recurrence ; Retrospective Studies ; Steroids

Rosai-Dorfman disease also known as sinus histiocytosis with massive lymphadenopathy is a benign, rare systemic disease characterized by a histiocyte proliferation which presents with lymphadenopathy. We report a case of a 4-year-old girl who presented with recurrent cervical lymphadenopathy with tenderness, without any other symptoms. After 1 month of medical treatment, her lymphadenopathy still remained, so we performed complete excision and biopsy. She was diagnosed on cytology as a case of Rosai-Dorfman disease. She responded well to become asymptomatic without recurrence by 1 month.
Biopsy ; Child ; Child, Preschool* ; Female* ; Histiocytes ; Histiocytosis ; Histiocytosis, Sinus* ; Humans ; Lymphatic Diseases ; Recurrence

Given that Kawasaki disease (KD) can cause cardiac complications, it is crucial to diagnose and treat the disease in the emergency department. We report a case of a 10-year-old girl who presented to the emergency department with cervical lymphadenopathy and fever. The initial diagnosis was retropharyngeal abscess based on computed tomography findings, but antibiotic therapy failed and she was subsequently diagnosed with KD. After 3 doses of intravenous immunoglobulin along with methylprednisolone and methotrexate, she was discharged on hospital day 21. The cervical lymphadenopathy suggesting retropharyngeal abscess might be an early sign of incomplete or intravenous immunoglobulin-resistant KD.
Child* ; Diagnosis* ; Emergency Service, Hospital ; Female* ; Fever* ; Humans ; Immunoglobulins ; Lymphatic Diseases* ; Methotrexate ; Methylprednisolone ; Mucocutaneous Lymph Node Syndrome* ; Neck Pain ; Retropharyngeal Abscess* ; Vasculitis

PURPOSE: Chloramphenicol and tetracycline are not recommended for treating scrub typhus in pediatric patients because of potential side effects, such as aplastic anemia or tooth discoloration. While clarithromycin has recently been used in adults, few reports have been published on its effects in pediatric patients. We report the clinical profiles of pediatric scrub typhus and the effects of clarithromycin on scrub typhus in children. METHODS: We retrospectively analyzed medical records of 56 children with scrub typhus who were admitted between 2004 and 2013 to Chonbuk National University Hospital, Jeonju, Korea. Cases were divided into 3 groups based on thetreatment drug (chloramphenicol, azithromycin, and clarithromycin). We compared their clinical manifestations and laboratory findings. RESULTS: All patients exhibited fever and rash. Other common clinical manifestations were eschars (66%), lymphadenopathy (48%), upper respiratory symptoms (42%), abdominal pain (32%), and hepatosplenomegaly (14%). Elevated levels of C-reactive protein, erythrocyte sedimentation rates, aspartate transaminase, and alanine transaminase were detected in 95%, 96%, 84%, and 77% of patients, respectively. Additionally, decreased platelet and white blood cell levels were observed in 43% and 36% of patients, respectively. There were no statistical differences between the treatment groups in mean age (P=0.114) or sex (P=0.507). However, time to defervescence after the treatments differed significantly, being the shortest in the clarithromycin group (P=0.019). All patients recovered without complications related to the disease or drugs. CONCLUSION: Clarithromycin was as effective as chloramphenicol and azithromycin in pediatric scrub typhus patients and may be used as a first-line treatment drug.
Abdominal Pain ; Adult ; Alanine Transaminase ; Anemia, Aplastic ; Aspartate Aminotransferases ; Azithromycin* ; Blood Platelets ; Blood Sedimentation ; C-Reactive Protein ; Child* ; Chloramphenicol* ; Clarithromycin* ; Exanthema ; Fever ; Humans ; Jeollabuk-do ; Korea ; Leukocytes ; Lymphatic Diseases ; Medical Records ; Retrospective Studies ; Scrub Typhus* ; Tetracycline ; Tooth Discoloration