Reticulohistiocytoma is a rare, benign histiocytic proliferation of the skin or soft tissue. A 5-month-old healthy girl visited our clinic for an enlarging nodule on the center of her right palm. The clinical differential diagnosis included xanthogranuloma and primary cutaneous CD4 positive small/medium T-cell lymphoma. Histopathology of the nodule showed abundant eosinophilic and glassy cytoplasm. The nuclei were round to oval shaped, with focal irregular nuclear membrane, and mitotic figures were absent. Immunohistochemical study determined that the cells were positive for CD68 but negative for CD1a. She was finally diagnosed with reticulohistiocytoma (solitary epithelioid histiocytoma).
Cytoplasm ; Diagnosis, Differential ; Eosinophils ; Female ; Histiocytosis, Non-Langerhans-Cell ; Humans ; Infant ; Lymphoma, T-Cell ; Nuclear Envelope ; Skin

Primary clinical features of rash and neurological complications due to varicella zoster virus (VZV) reactivation are rare in a healthy population, especially in immunocompetent children. Early diagnosis and prompt treatment are delayed often due to their rarity. We present four immunocompetent children with VZV reactivation resulting in aseptic meningitis and herpes zoster affecting multiple cranial and spinal nerves. We reviewed the clinical manifestations, laboratory findings, treatment options and outcome of aseptic meningitis associated VZV reactivation. All patients presented with the typical skin lesion of VZV reactivation and definitive laboratory findings of central nervous system infection, without systemic inflammation. Initial manifestations of VZV reactivation included Ramsay Hunt syndrome, herpes zoster ophthalmicus, and herpes zoster affecting the left thoracic dermatomes 4–5. Intravenous acyclovir was administered and all patients recovered fully without any significant sequelae. VZV reactivation can lead to various neurological complications in immunocompetent children. Early recognition and treatment with acyclovir are important for improving the outcome of neurologic complications of VZV reactivation.
Acyclovir ; Central Nervous System Infections ; Chickenpox ; Child* ; Early Diagnosis ; Exanthema ; Herpes Zoster Ophthalmicus ; Herpes Zoster Oticus ; Herpes Zoster* ; Herpesvirus 3, Human ; Humans ; Inflammation ; Meningitis, Aseptic* ; Skin ; Spinal Nerves

PURPOSE: The aim of this study is to evaluate the prevalence and risk factors of seizure aggravation of adjunctive levetiracetam therapy in children with epilepsy. METHODS: We retrospectively identified 125 children (0.3–18 years) with epilepsy who were newly treated with adjunctive levetiracetam therapy from November 2008 to July 2014 in Pusan National University Hospital, and 44 patients were excluded according to the exclusion criteria. Aggravation was diagnosed if the seizure frequency increased by more than 50% of baseline or there were new types of seizures after 1 month of adjunctive levetiracetam therapy. RESULTS: Eighty-one patients (male:female, 44:37) were enrolled, including 27 (33.5%) with generalized seizures and 54 (66.7%) with focal seizures. Twelve patients (14.8%) exhibited seizure aggravation and 69 patients (85.2%) had improvement or no change after 1 month of levetiracetam therapy. Eleven patients (91.7%) in seizure aggravation group and 16 patients (23.2%) in non-seizure aggravation group had generalized seizures, with aggravation significantly more frequent in patients with generalized seizures (P < 0.001). Other factors such as age at diagnosis, age at adding levetiracetam, sex, baseline seizure frequency, etiology, electroencephalography and magnetic resonance imaging abnormalities, and concomitant drug use were not identified as risk factors. CONCLUSION: Although levetiracetam is an effective antiepileptic drug in children with epilepsy, adjunctive levetiracetam therapy was associated with worsening of seizures in 14.8 % of included patients, especially those with generalized seizures. Careful monitoring for increased seizure frequency or the onset of a new type of seizures is advised for patients prescribed levetiracetam add-on treatment.
Anticonvulsants ; Busan ; Child* ; Diagnosis ; Electroencephalography ; Epilepsy* ; Epilepsy, Generalized ; Humans ; Magnetic Resonance Imaging ; Prevalence ; Retrospective Studies ; Risk Factors ; Seizures*

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

Anaplastic lymphoma kinase (ALK) fusion is a common mechanism underlying pathogenesis of non-small cell lung carcinoma (NSCLC) where these rearrangements represent important diagnostic and therapeutic targets. In this study, we found a new ALK fusion gene, SEC31A-ALK, in lung carcinoma from a 53-year-old Korean man. The conjoined region in the fusion transcript was generated by the fusion of SEC31A exon 21 and ALK exon 20 by genomic rearrangement, which contributed to generation of an intact, in-frame open reading frame. SEC31A-ALK encodes a predicted fusion protein of 1,438 amino acids comprising the WD40 domain of SEC31A at the N-terminus and ALK kinase domain at the C-terminus. Fluorescence in situ hybridization studies suggested that SEC31A-ALK was generated by an unbalanced genomic rearrangement associated with loss of the 3'-end of SEC31A. This is the first report of SEC31A-ALK fusion transcript in clinical NSCLC, which could be a novel diagnostic and therapeutic target for patients with NSCLC.
Adenocarcinoma* ; Amino Acids ; Exons ; Fluorescence ; Humans ; In Situ Hybridization ; Lung Neoplasms ; Lung* ; Lymphoma ; Middle Aged ; Open Reading Frames ; Phosphotransferases ; Translocation, Genetic

PURPOSE: Bell's palsy is characterized by sudden onset of unilateral facial weakness. The use of corticosteroids for childhood Bell's palsy is controversial. This study aimed to identify clinical characteristics, etiology, and laboratory findings in childhood Bell's palsy, and to evaluate the efficacy of corticosteroid treatment. METHODS: We conducted a retrospective analysis of children under 19 years of age treated for Bell's palsy between January 2009 and June 2017, and followed up for over 1 month. Clinical characteristics, neuroimaging data, laboratory findings, treatments, and outcomes were reviewed. Patients with Bell's palsy were divided into groups with (group 1) and without (group 2) corticosteroid treatment. Differences in onset age, sex, laterality, infection and vaccination history, degree of facial nerve palsy, and prognosis after treatment between the groups were analyzed. RESULTS: One hundred patients were included. Mean age at presentation was 7.4±5.62 years. A total of 73 patients (73%) received corticosteroids with or without intravenous antiviral agents, and 27 (27%) received only supportive treatment. There was no significant difference in the severity, laboratory findings, or neuroimaging findings between the groups. Significant improvement was observed in 68 (93.2%) and 26 patients (96.3%) in groups 1 and 2, respectively; this rate was not significantly different between the groups (P=0.48). CONCLUSION: Childhood Bell's palsy showed good prognosis with or without corticosteroid treatment; there was no difference in prognosis between treated and untreated groups. Steroid therapy in childhood Bell's palsy may not significantly improve outcomes.
Adrenal Cortex Hormones ; Age of Onset ; Antiviral Agents ; Bell Palsy* ; Child* ; Facial Nerve ; Facial Paralysis ; Humans ; Neuroimaging ; Paralysis ; Prognosis ; Retrospective Studies ; Vaccination

Herpes simplex virus (HSV) is a common pathogen, that causes a broad spectrum of diseases, ranging from minor skin infections to severe encephalitis and widespread infections. Acute retinal necrosis (ARN), one of the most serious manifestations of HSV infection, is defined as a rapidly progressing necrotizing retinopathy that presents discrete areas of circumferential retinal necrosis, along with signs of uveitis, vitreitis, and retinal vasculitis. We encountered a case of a female infant, born at 33 weeks of gestation with a body weight at birth of 2,080 g, who had ARN and encephalomalacia due to HSV infection. ARN associated with HSV infection should be suspected when nonspecific retinal exudates are observed in neonates, especially preterm infants.
Body Weight ; Encephalitis ; Encephalomalacia ; Exudates and Transudates ; Female ; Herpes Simplex ; Herpesvirus 2, Human ; Humans ; Infant ; Infant, Newborn ; Infant, Premature ; Necrosis ; Parturition ; Pregnancy ; Retinal Necrosis Syndrome, Acute ; Retinal Vasculitis ; Retinaldehyde ; Simplexvirus ; Skin ; Uveitis

Magnetic resonance imaging (MRI) is recommended for patients with epileptic seizures to rule out an underlying focal lesion. However, abnormalities in idiopathic generalized epilepsy, including childhood absence epilepsy, cannot usually be identified using brain imaging modalities such as MRI. Peri-ictal MRI abnormalities have been most commonly reported secondary to status epilepticus and are rarely observed in patients with focal seizures and generalized tonic-clonic seizures. Transient peri-ictal MRI abnormalities in absence epilepsy are extremely rare. A five-year-old girl presented with a three-day history of absence seizures that persisted despite continued treatment with sodium valproate. Electroencephalography showed bursts of generalized 3-Hz spike-and-wave discharges, during and after hyperventilation. Abnormal cortex thickening in the left perisylvian region was detected on T2-weighted brain MRI, and cortical dysplasia or a tumor was suspected. The patient started treatment with lamotrigine and was seizure-free after one month. The abnormal MRI lesion was completely resolved at the two-month follow-up. We report on a patient with childhood absence epilepsy and reversible brain MRI abnormalities in the perisylvian region. To our knowledge, this is the first report of transient MRI abnormalities after absence seizures. Transient peri-ictal MRI abnormalities should be considered for differential diagnosis in patients with absence seizures and a focal abnormality on brain MRI.
Brain ; Diagnosis, Differential ; Electroencephalography ; Epilepsy ; Epilepsy, Absence* ; Epilepsy, Generalized ; Female ; Follow-Up Studies ; Humans ; Hyperventilation ; Magnetic Resonance Imaging* ; Malformations of Cortical Development ; Neuroimaging ; Seizures ; Status Epilepticus ; Valproic Acid