Background: Psoriasis is a chronic inflammatory skin disease. Despite the current focus on T cells in the pathogenesis of psoriasis, keratinocytes within the psoriatic epidermis are also abnormal in many respects including excessive production of antimicrobial peptides (AMPs). Recently, several studies have used imiquimod (IMQ)- and interleukin (IL)-23-induced mouse models to explore the immunological significance of psoriasis. However, the expression of AMPs in these models remains unclear. Objective: In this study, we evaluated the protein and mRNA expression of AMPs, including cathelicidin-related antimicrobial peptides (CRAMP), mouse β-defensin 3 (mBD3), and psoriasin (S100 calcium-binding protein A7, S100A7) in IMQ- and IL-23-induced psoriasiform mouse models. In addition, we investigated whether ustekinumab, an inhibitor of IL-12 and IL-23, reduces the expression levels of AMPs in these mouse models. Methods: We used IMQ- and IL-23-induced psoriasiform mouse models. Gene and protein expression levels of AMPs were evaluated using quantitative real-time polymerase chain reaction and immunofluorescence staining, respectively. Results: We found that the protein and mRNA expression levels of CRAMP, mBD3, and S100A7 were increased in both mouse models. Ustekinumab decreased AMP expression levels in the two mouse models. Conclusion These data showed that the elevated expression of AMPs in the epidermis decreased following ustekinumab treatment in both psoriasiform mouse models. Therefore, AMP expression levels may be used as an indicator of treatment efficacy.

Purpose: Given the importance of evaluating the severity of overactive bladder (OAB) symptoms and outcomes after treatment, several questionnaires have been developed to evaluate OAB patients. However, only limited questionnaires are available in Korea for use with Korean patients. Therefore, this study aimed to develop Korean versions of OAB questionnaires through a rigorous linguistic validation process. Methods: The Indevus Urgency Severity Scale, Urgency Perception Scale, Urgency Severity Scale, and Patient Perception of Intensity of Urgency Scale underwent translation and linguistic validation. The linguistic validation procedure consisted of permission for translation, forward translations, reconciliation, back-translation, cognitive debriefing, and proofreading. Two independent bilingual translators translated the original version of each questionnaire, and a panel then discussed and reconciled the 2 initial translations. Next, a third independent bilingual translator performed a backward translation of the reconciled version into English. Five Korean patients diagnosed with OAB were interviewed for cognitive debriefing. Results: Each item of the questionnaires was translated into 2 Korean versions in the forward translation process. Terms such as ‘urgency’ and ‘wetting’ were translated into ordinary language by the translators and adjusted by the panel members to more conceptually equivalent terms in a medical context. In the back-translation process, the panel made a few changes regarding details based on a comparison of the back-translated and original versions. During the cognitive debriefing process, 5 patients provided a few pieces of feedback on the naturalness of the wording of the questionnaires, but generally agreed on the translated terms. Conclusions In this study, the panel produced a successful linguistic validation of Korean versions of multiple OAB questionnaires, which can be utilized to evaluate the severity and treatment outcomes of OAB.

Pancreatitis, panniculitis, and polyarthritis (PPP) syndrome is a rare but critical disease with a high mortality rate. The diagnostic dilemma of PPP syndrome is the fact that symptoms occur unexpectedly. A 48-year-old man presented with fever and painful swelling of the left foot that was initially mistaken for cellulitis and gouty arthritis. The diagnosis of PPP syndrome was made based on the abdominal CT findings and elevated pancreatic enzyme levels, lobular panniculitis with ghost cells on a skin biopsy, and polyarthritis on a bone scan. The pancreatitis and panniculitis disappeared spontaneously over time, but the polyarthritis followed its own course despite the use of anti-inflammatory agents. In addition to this case, 30 cases of PPP syndrome in the English literature were reviewed. Most of the patients had initial symptoms other than abdominal pain, leading to misdiagnosis. About one-third of them were finally diagnosed with a pancreatic tumor, of which pancreatic acinar cell carcinoma was the most dominant. They showed a mortality rate of 32.3%, associated mainly with the pancreatic malignancy. Therefore, PPP syndrome should be considered when cutaneous or osteoarticular manifestations occur in patients with pancreatitis. Active investigation and continued observations are needed for patients suspected of PPP syndrome.
Abdominal Pain ; Anti-Inflammatory Agents ; Arthritis ; Arthritis, Gouty ; Biopsy ; Carcinoma, Acinar Cell ; Cellulitis ; Diagnosis ; Diagnostic Errors ; Fever ; Foot ; Humans ; Middle Aged ; Mortality ; Pancreatic Neoplasms ; Pancreatitis ; Panniculitis ; Skin ; Tomography, X-Ray Computed

Pancreatitis, panniculitis, and polyarthritis (PPP) syndrome is a rare but critical disease with a high mortality rate. The diagnostic dilemma of PPP syndrome is the fact that symptoms occur unexpectedly. A 48-year-old man presented with fever and painful swelling of the left foot that was initially mistaken for cellulitis and gouty arthritis. The diagnosis of PPP syndrome was made based on the abdominal CT findings and elevated pancreatic enzyme levels, lobular panniculitis with ghost cells on a skin biopsy, and polyarthritis on a bone scan. The pancreatitis and panniculitis disappeared spontaneously over time, but the polyarthritis followed its own course despite the use of anti-inflammatory agents. In addition to this case, 30 cases of PPP syndrome in the English literature were reviewed. Most of the patients had initial symptoms other than abdominal pain, leading to misdiagnosis. About one-third of them were finally diagnosed with a pancreatic tumor, of which pancreatic acinar cell carcinoma was the most dominant. They showed a mortality rate of 32.3%, associated mainly with the pancreatic malignancy. Therefore, PPP syndrome should be considered when cutaneous or osteoarticular manifestations occur in patients with pancreatitis. Active investigation and continued observations are needed for patients suspected of PPP syndrome.
Abdominal Pain ; Anti-Inflammatory Agents ; Arthritis ; Arthritis, Gouty ; Biopsy ; Carcinoma, Acinar Cell ; Cellulitis ; Diagnosis ; Diagnostic Errors ; Fever ; Foot ; Humans ; Middle Aged ; Mortality ; Pancreatic Neoplasms ; Pancreatitis ; Panniculitis ; Skin ; Tomography, X-Ray Computed

Olmsted syndrome (OS) is a rare congenital skin disorder characterized by severe palmoplantar and periorificial keratoderma, alopecia, onychodystrophy, and severe pruritus. Recently, pathogenic ‘gain-of-function‘ mutations of the transient receptor potential vanilloid 3 gene (TRPV3), which encodes a cation channel involved in keratinocyte differentiation and proliferation, hair growth, inflammation, pain and pruritus, have been identified to cause OS. Due to the rarity, the pattern of inheritance of OS is still unclear. We report a case of OS in a 3-year-old Korean girl and its underlying gene mutation. The patient presented with a disabling, bilateral palmoplantar keratoderma with onychodystrophy. She also exhibited pruritic eczematous skin lesions around her eyes, ears and gluteal fold. Genetic analysis identified a heterozygous p.Gly568Val missense mutation in the exon 13 of TRPV3. To our knowledge, this is the first case of OS in the Korean population showing a missense mutation p.Gly573Ser.
Abnormalities, Multiple/*genetics ; Base Sequence ; Child, Preschool ; Female ; Heterozygote ; Humans ; Keratoderma, Palmoplantar/genetics ; Lipid Droplets/ultrastructure ; Mutation, Missense/*genetics ; Skin/pathology/ultrastructure ; Syndrome ; TRPV Cation Channels/*genetics

BACKGROUND AND PURPOSE: Perampanel is the first α-amino-3-hydroxy-5-methyl-4-isoxazole-propionic acid (AMPA)-receptor antagonist developed to treat epilepsy. The effects of either rapid or slow dose titration on adverse events remain to be elucidated. METHODS: Eighty-five patients received perampanel between March 2016 and August 2016. Patients were divided into two groups according to their dosing schedule: rapid dose titration (2-mg increments at intervals of 1 to 2 weeks) and slow dose titration (2-mg increments at intervals of at least 3 weeks). Seizure frequency and adverse events were analyzed over 3 months. RESULTS: Adverse events were reported by 47 (58%) of the 81 patients analyzed, with 12 (15%) patients discontinuing perampanel due to adverse events. Common adverse events included dizziness (n=30, 37%), aggressive mood and behavior (n=19, 24%), gait disturbance (n=16, 20%), and sleep problems (n=10, 12.4%). The overall adverse events were similar in the slow-titration group (38 of 61 patients) and the rapid-titration group (8 of 20 patients, p=0.081). However, none of the 20 patients in the slow-titration group experienced gait disturbance, compared with 16 of the 61 patients in the rapid-titration group (p=0.009), while appetite change was experienced by 4 patients in the slow-titration group but only 1 in the rapid-titration group (p=0.003). No relationship was noted between adverse events and the maximum dose of perampanel (p=0.116). Sex differences were observed, with the response to perampanel being better and the rate of adverse events being higher in females (p=0.015 and p=0.046, respectively). CONCLUSIONS: Slow titration of perampanel may reduce perampanel-related adverse events.
Appetite ; Appointments and Schedules ; Dizziness ; Drug Resistant Epilepsy* ; Epilepsy ; Female ; Gait ; Humans ; Seizures ; Sex Characteristics

PURPOSE: Atopic dermatitis (AD) is a chronic, relapsing eczematous inflammatory skin disease. Mutations in the filaggrin gene (FLG) are major predisposing factors for AD. Ethnic differences exist between Asian and European populations in the frequency and spectrum of FLG mutations. Moreover, a distinct set of FLG mutations has been reported in Asian populations. The aim of this study was to examine the spectrum of FLG mutations in Koreans with AD. We also investigated the association of FLG mutations and clinical features of AD and compared the Korean FLG landscape with that of other East Asian countries. MATERIALS AND METHODS: Seventy Korean patients with AD were enrolled in this study. Fourteen FLG mutations previously detected in Korean, Japanese, and Chinese patients were screened by genotyping. RESULTS: Four FLG null mutations (3321delA, K4022X, S3296X, and S2889X) were identified in eleven patients (15.7%). The most commonly detected mutations in Korean patients with AD were 3321delA (n=6, 9.1%) and K4022X (n=3, 4.5%). FLG mutations were significantly associated with elevated IgE (≥200 KIU/L and/or MAST-CLA >3+, p=0.005), palmar hyperlinearity (p<0.001), and a family history of allergic disease (p=0.021). CONCLUSION: This study expanded our understanding of the landscape of FLG mutations in Koreans and revealed an association between FLG mutations and AD phenotype.
Asian Continental Ancestry Group ; Causality ; Dermatitis, Atopic* ; Humans ; Immunoglobulin E ; Phenotype ; Skin Diseases

The present study evaluated the effects of a mixture of Galla rhois and Cinnamomum cassia extracts (GCE) (1 : 1, w/w) on susceptibility to the colonization of Campylobacter (C.) jejuni in broilers. Eighty two-week-old broilers (n = 20 per group) were used to estimate the efficacy of GCE against C. jejuni infection via drinking water. Antibacterial activity testing revealed that the minimum bactericidal concentration of GCE against C. jejuni was 2.5 mg/mL. Broilers challenged with C. jejuni were administered 0.0 (Non-GCE), 2.5 (GCE-2.5), 5.0 (GCE-5.0) and 10.0 g/L (GCE-10) GCE for 7 days, and the cecal contents were collected from five broilers per group on the 1st, 3rd, 5th, and 7th day post-treatment. On day 3 post-administration, the number of C. jejuni in GCE-5.0 (p < 0.05) and GCE-10 (p < 0.01) was significantly decreased relative to Non-GCE, while on day 7 those in all GCE-treated groups were significantly decreased compared to the Non-GCE group (p < 0.001). Hematological and blood biochemical analysis revealed no significant differences in parameters between the Non-GCE and GCE-treated groups. Based on the results of the present study, GCE was identified as a safe and alternative candidate to suppress C. jejuni colonization in broilers.
Campylobacter jejuni* ; Campylobacter* ; Chickens* ; Cinnamomum aromaticum* ; Cinnamomum* ; Colon* ; Drinking Water

No abstract available.
Asian Continental Ancestry Group* ; Humans ; Sequence Deletion*