Idiopathic inflammatory myopathies (IIM) are a heterogeneous group of autoimmune muscle diseases with systemic involvement. Patients with IIM present with varying degrees of muscle disease, cutaneous manifestations, and internal organ involvement. The diagnosis and classification of IIM is based primarily on the classification system composed of clinical features, laboratory value and muscle biopsy. In addition, the identification and characterization of myositis-related autoantibodies can help diagnosis and classification. Recently, many studies have also demonstrated that the physician can define the clinical syndromes, establish treatment strategy and predict outcomes based on the patients' myositis-specific autoantibodies (MSA) and myositis-associated antibodies (MAA) profiles. MSAs are found exclusively in IIMs and facilitate the identification of subsets of patients with relatively homogeneous clinical features. MAAs are frequently found in association with other MSA; however, they may also be detected in various connective diseases.
Antibodies ; Antibodies, Antinuclear ; Autoantibodies ; Biopsy ; Classification ; Dermatomyositis ; Diagnosis ; Humans ; Myositis ; Polymyositis

Retroperitoneal fibrosis (RPF) is a rare disease characterized by marked fibro-inflammatory tissue in the retroperitoneum. Approximately 70% of cases of RPF are idiopathic, while the rest can be secondary to several other causes. The diagnosis is mainly obtained by imaging modalities such as computed tomography (CT). However, histological examination should be considered when the clinical manifestations and imaging studies suggest malignancy. In particular, in malignant diseases with retroperitoneal metastases, abnormal collagen plaques are formed from an exuberant desmoplastic response, which may not be distinguishable from RPF on CT scans. Therefore, even if CT suggests RPF, biopsy is essential to identify malignant disease because it typically results in a fatal prognosis. Here, we report a case of metastatic adenocarcinoma of unknown primary site that developed only in the retroperitoneum and was initially diagnosed as RPF based on CT findings.
Adenocarcinoma ; Biopsy ; Collagen ; Diagnosis ; Neoplasm Metastasis ; Neoplasms, Unknown Primary ; Prognosis ; Rare Diseases ; Retroperitoneal Fibrosis* ; Tomography, X-Ray Computed

PURPOSE: The function of regulatory B lymphocytes is known to be abnormal in inflammatory diseases. However, a recent study indicates that IL-10+ B cells seem to be expanded in rheumatoid arthritis (RA). Therefore, the state of IL-10+ B cells in the peripheral blood from RA patients and healthy controls were investigated. MATERIALS AND METHODS: CD19+ cells in peripheral blood mononuclear cells were purified from blood samples of RA patients and age and gender-matched healthy controls, and stimulated with CD40 ligand and CpG for 48 hours. Then, intracellular IL-10 in CD19+ cells was analyzed using flow cytometry. RESULTS: There was no significant difference in the proportion of IL-10+ B cells between 10 RA patients and 10 healthy controls (RA, 0.300+/-0.07 vs. healthy control 0.459+/-0.07, p=0.114). The proportion of induced IL-10+ B cells to total B cells in RA patients was significantly higher than those in controls (RA, 4.44+/-3.44% vs. healthy control 2.44+/-1.64%, p=0.033). However, the proportion of IL-10+ B cells to total B cells correlated negatively with disease activity in RA patients (r=-0.398, p=0.040). Erythrocyte sedimentation rate or C-reactive protein or medication was not associated with the proportion of IL-10+ B cells. CONCLUSION: The proportion of induced IL-10+ B cell increased in RA patients compared to healthy control, however, negatively correlated with disease activity in RA.
Adult ; Aged ; Antigens, CD19/metabolism ; Arthritis, Rheumatoid/blood/*immunology/pathology ; B-Lymphocytes, Regulatory/metabolism/*physiology ; Biological Markers/blood ; Female ; Humans ; Interleukin-10/metabolism ; Male ; Middle Aged ; Severity of Illness Index

PURPOSE: We aimed to describe the difference in the epidemiologic and clinical characteristics by age groups of the children with hand injuries requiring surgery who visited the emergency department (ED) of a community hospital that runs a hand center. METHODS: We reviewed 388 consecutive children with hand injuries requiring surgery, aged < 16 years, who visited the ED from January 2011 through September 2016. Information was obtained regarding age and gender of the children, seasonal and daily distribution of the visits, location, cause, site, and level of the injury, the diagnosis, and presence of serious injury. The children were classified into 3 age groups; toddlers (0 to 3 years), preschoolers (4 to 6 years), and schoolers (7 to 15 years). Severe injury was defined as amputation or crush injury. RESULTS: Mean age of the children was 7.4 ± 5.0 years and boys accounted for 65.7%. The most frequent visits occurred during the weekend (53.1%) and in spring (30.7%), and most children visited the ED with injuries that occurred at home and indoors (55.2% and 79.9%, respectively). The most common cause, site, and level of the injury were sharp object (34.8%), fingers other than the thumb and index finger (64.7%), and the distal phalanx (46.7%), respectively. In the toddler group, domestic, indoor, door-related, and distal phalanx injuries were more common than in the schooler group (P < 0.001). Physical contact or sharp object-related injuries increased with increasing age (P < 0.001). Severe injuries were more common among the toddlers than the schoolers (P < 0.001). CONCLUSION: In the toddler group, domestic, indoor, door-related, distal phalanx, and severe injuries were more common than in the schooler group. These characteristics by age groups would aid in preventing hand injury in children, especially toddlers.
Amputation ; Child* ; Diagnosis ; Emergencies* ; Emergency Service, Hospital* ; Fingers ; Hand Injuries* ; Hand* ; Hospitals, Community ; Humans ; Pediatrics ; Seasons ; Thumb

Dermatomyositis (DM) and polymyositis (PM) are representative idiopathic inflammatory myopathies characterized by symmetric and progressive proximal muscle weakness. Especially, DM is identified by characteristic skin lesions and has many extramuscular manifestations including various cardiac abnormalities, interstitial lung disease, and malignancy. However, involvement of peripheral nervous system in DM/PM is very rare and less known. The term “Neuromyositis” was introduced by Senator in 1893 to describe the concomitant involvement of the peripheral nervous system in DM/PM. Since then, a very few cases of neuromyositis have been reported mainly in the United States and Europe. Therefore, the pathogenetic mechanism and disease progression are unclear. In recent years, a few more cases were reported in Asia, specifically, China and Japan; however, none in Korea. Here, we describe a case of DM-associated neuromyositis in a 42-year-old man in Korea and review previous publications through literature research.
Adult ; Asia ; China ; Dermatomyositis ; Disease Progression ; Electromyography ; Europe ; Humans ; Japan ; Korea ; Lung Diseases, Interstitial ; Muscle Weakness ; Myositis ; Neural Conduction ; Peripheral Nervous System ; Peripheral Nervous System Diseases ; Polymyositis ; Skin ; United States

The elastin metabolism in systemic sclerosis (SSc) has been known to be abnormal. The authors investigated relationship between the clinical manifestations of systemic sclerosis (SSc) and serum levels of soluble elastin-derived peptide (S-EDP) and anti-elastin antibodies. Serum samples were obtained from 79 patients with SSc and 79 age- and sex-matched healthy controls. Concentrations of serum S-EDP and anti-elastin antibodies were measured by ELISA. The serum concentrations of S-EDP in SSc patients were significantly higher than in healthy controls (median, 144.44 ng/mL vs 79.59 ng/mL, P < 0.001). Serum EDP concentrations were found to be correlated with disease duration in SSc (P = 0.002) and particularly in diffuse cutaneous SSc (P = 0.005). Levels of anti-elastin antibodies were found to be more elevated in SSc patients than in healthy controls (median, 0.222 U vs 0.191 U, P = 0.049), more increased in diffuse cutaneous SSc than limited cutaneous SSc (median, 0.368 U vs 0.204 U, P = 0.031). In addition, levels of anti-elastin antibodies were also found to be negatively associated with presence of anti-centromere antibody (P = 0.023). The S-EDP levels were not found to be correlated with levels of anti-elastin antibodies. The increased S-EDP and anti-elastin antibody levels and association with clinical and laboratory characteristics may reflect the abnormal metabolism in SSc.
Adult ; Antibodies, Anti-Idiotypic/*blood/immunology ; Centromere/immunology ; Elastin/*blood/immunology ; Enzyme-Linked Immunosorbent Assay ; Female ; Humans ; Male ; Middle Aged ; Peptides/*blood/immunology ; Scleroderma, Systemic/*metabolism/pathology

Hypertrophic osteoarthropathy is a syndrome characterized by periosteal new bone formation, arthritis, and clubbing of the fingers and toes. The majority of cases occur secondarily to the conditions associated with pulmonary, cardiac, gastrointestinal disorders or other systemic diseases. There are many cases with malignancy worldwide. We report the first patient who had hypertrophic osteoarthropathy due to metastatic cancer after surgical removal for mucoepidermoid carcinoma of the parotid gland.
Arthritis ; Carcinoma, Mucoepidermoid* ; Fingers ; Humans ; Lung* ; Neoplasm Metastasis* ; Osteogenesis ; Parotid Gland* ; Toes

Currently, infliximab is given for disease control for active rheumatoid arthritis (RA) patients despite methotrexate treatment. However, the efficacy and safety of infliximab in Korean patients has not been assessed appropriately. Therefore, we performed placebo-controlled, double-blind, randomized study and extension study. One-hundred forty-three patients with active RA were randomized to receive placebo or infliximab 3 mg/kg intravenously at week 0, 2, 6, 14, and 22 with methotrexate maintenance. Primary endpoint was American College of Rheumatology 20% improvement criteria (ACR20) at 30 week. After the clinical trial, patients on placebo (Group 1) and patients on infliximab who showed ACR20 response (Group 2) were treated with infliximab through another 84 week for evaluation of safety. During clinical trial, patients in infliximab group showed higher ACR20 at week 30 than patients in placebo group (50.1% vs 30.6%, P=0.014). A total of 92 patients participated in the extension study. The maintenance rate of infliximab was 62.0% at 84 weeks of extension study. The overall rate of adverse events was not different between Group 1 and Group 2. In Korean patients with active RA despite methotrexate treatment, infliximab in combination with methotrexate is effective and the long-term treatment with infliximab is well tolerated. (ClinicalTrials.gov No. NCT00202852, NCT00732875)
Adult ; Aged ; Antibodies, Monoclonal/*therapeutic use ; Antirheumatic Agents/*therapeutic use ; Arthritis, Rheumatoid/*drug therapy ; Double-Blind Method ; Drug Therapy, Combination ; Female ; Humans ; Male ; Methotrexate/*therapeutic use ; Middle Aged ; Placebo Effect ; Republic of Korea ; Severity of Illness Index ; Time Factors ; Treatment Outcome

Objective: Patients presenting with fingertip skin defect injuries without exposed bone can avail of two treatment options at the emergency department (ED). This study compared outcomes between dressing and composite graft (CG) using skin stump for patients visiting the ED with fingertip skin defect injuries without exposed bone. Methods: This was a single-center, retrospective, observational study. We reviewed 244 patients with fingertip skin defect injuries without exposed bone who visited the ED from September 2018 to February 2021. We compared the outcomes of the patients who were treated by CG using skin stump and those who received a dressing in the ED. Results: In all, 142 patients were treated by CG using skin stump, and 102 patients were given a dressing only. In the CG group, good outcomes were obtained in 140 patients, whereas additional skin graft treatment was required for two patients with bad outcomes. In the dressing group, 81 patients had good outcomes and 21 patients had bad outcomes which required additional skin graft treatment. Conclusion Results of our study revealed that compared to traditional dressing, ED treatment for fingertip skin defects without exposed bone showed good outcomes when administered CG using skin stump. Hence, we recommend that instead of simple dressing, CG using skin stump is the preferred mode of treatment for patients presenting in the ED with fingertip skin defect injuries without exposed bone.

Background: Pilomatrixoma is a benign tumor that originates from the hair follicle matrix. It usually presents as a hard, slow growing, solitary mass that can be easily misdiagnosed as other skin masses. The aim of this study was to clinically analyze a case series of pilomatrixoma in pediatric patients from Korea. Methods: A total of 165 pediatric patients from 2011 to 2018 with a histological diagnosis of pilomatrixoma were included. A retrospective review was performed using the electronic medical records, including patient demographics, number and location of the mass, clinical and imaging presentation, and postoperative outcomes. Results: There were 61 male and 104 female patients with 152 solitary and 13 multiple pilomatrixomas. Among solitary pilomatrixomas, the lesion commonly occurred in the head and neck (84.2%), followed by upper limbs (11.2%), lower limbs (3.3%), and trunk (1.3%). The pilomatrixoma lesion presented as the following types based on our clinical classification: mass (56.02%), pigmentation (25.31%), mixed (12.65%), ulceration (4.82%), and keloid-like (1.2%). Ultrasonography showed a high positive predictive value (95.56%). There were no specific complications observed except for two cases of recurrence. Conclusion Pilomatrixoma has various clinical feature presentations and commonly occurs in the head and neck. Ultrasonography is a helpful diagnostic tool. Surgical removal of the lesion is the main treatment method with a low recurrence rate.