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Nevus sebaceus is a hamartomatous lesion characterized by epidermal, follicular, sebaceus, and apocrine gland abnormalities. Approximately 25% of affected individuals may develop benign or malignant secondary neoplasms within the preceding nevus sebaceus. Primary cutaneous apocrine carcinoma (PCAC) is a rare malignant skin tumor affecting elderly adults in their sixth decade of life. Histologically, PCAC appears as a dermal tumor displaying apocrine differentiation with decapitation secretion and malignant features. Secondary malignancy arising from nevus sebaceus is a rare complication, especially for apocrine carcinoma. To date, approximately 200 cases of PCAC have been reported in the literature, and only a few cases have developed PCAC on the scalp. Very few cases (approximately only 12) of PCACs developing in nevus sebaceus have been reported. Here, we report an extremely rare case of the coexistence of PCAC and syringocystadenoma papilliferum arising within nevus sebaceus of the scalp.
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Pilar leiomyoma or piloleiomyoma is a benign neoplasm of the smooth muscle arising from the arrector pili muscle. It manifests as brown to red firm papulonodules with sites of predilection being the face, trunk, and extensor surfaces of the extremities. Histologically, the lesions exhibit ill-defined dermal tumors with interlacing fascicles of spindle cells. Some genodermatoses are characterized by the development of visceral tumors and cutaneous leiomyomatosis such as Reed’s syndrome, and hereditary leiomyomatosis and renal cell cancer (HLRCC). A 55-year-old male presented with reddish-brown papules and nodules on the face and upper back, accompanied by sharp episodic pain on the face. He had undergone nephrectomy for renal cancer 9 years ago, and his younger brother had similar cutaneous manifestation. Histopathologic findings were consistent with pilar leiomyoma, showing bundles of smooth muscle tumors in the dermis. Based on the clinical information including clinical features, past medical history, and family history, HLRCC was highly suspected.To confirm the diagnosis, whole exome sequencing was performed using peripheral blood, which revealed a novel point mutation (c.739G>A, p.Glu247Lys) in the fumarate hydratase (FH) gene. We describe a confirmed case of HLRCC, which is a genetic disorder with a potential to cause visceral cancers, which dermatologists might overlook as a benign condition.
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Rubinstein-Taybi syndrome (RSTS) is an extremely rare genetic disorder affecting multi-organ systems. A tendency to form keloid is one of the common dermatologic manifestations.We describe a 23-year-old female presented with extensive keloids which developed spontaneously. She had typical facial features, broad thumbs, and dental defects, which were suspicious features of genetic syndrome. Direct sequencing for cyclic-AMP-regulated enhancer binding protein revealed a novel mutation. So far, 23 cases of RSTS have been reported in Korean literature. To the best of our knowledge, this is the first report in Korea to describe confirmed case of RSTS with extensive keloids as a chief manifestation.
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Background@#Alopecia areata (AA) is a chronic disease with an unpredictable disease course and severe psychological impact. @*Objective@#To provide evidence- and consensus-based insights regarding the treatment of patients with AA in Korea. @*Methods@#We searched for relevant studies on the topical and device-based treatment of AA in the literature from inception until May 2021. Evidence-based recommendations were also prepared. The evidence for each statement was graded and classified according to the strength of the recommendations. Hair experts from the Korean Hair Research Society (KHRS) voted on the statements, and an agreement of 75% or greater was considered as consensus. @*Results@#Currently, there remains a scarcity of topical treatments, which is supported by robust evidence from a number of high-quality randomized controlled trials. Current evidence supports the efficacy of topical corticosteroids, corticosteroid intralesional injection, and contact immunotherapy in AA patients. Topical corticosteroids and contact immunotherapy are recommended for pediatric AA. A consensus was achieved in 6 out of 14 (42.8%), and 1 out of 5 (20.0%) statements pertaining to topical and device-based treatments in AA, respectively. The expert consensus was from a single country, and the study may not cover all the treatments used. @*Conclusion@#The present study provides up-to-date, evidence-based treatment guidelines for AA based on the consensus reached among experts after considering regional healthcare circumstances, adding diversity to the previous guidelines.
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Background@#Alopecia areata (AA) is a chronic disease with an unpredictable course and can have a severe psychological impact on an individual. @*Objective@#To provide evidence and consensus-based statements regarding the treatment of patients with AA in Korea. @*Methods@#We searched for relevant studies from inception to May 2021 regarding the systemic treatment of AA. Evidence-based recommendations were also prepared. The evidence for each statement was graded and classified according to the strength of the recommendations. Hair experts from the Korean Hair Research Society (KHRS) voted on the statement, and an agreement of 75% or greater was considered as having reached consensus. @*Results@#Current evidence supports the efficacy of systemic corticosteroids, oral cyclosporine monotherapy or combination with systemic corticosteroids, and oral Janus kinase inhibitors in severe AA patients. Systemic steroids may be considered for pediatric patients with severe AA. A consensus was achieved in three out of nine (33.3%), and one out of three (33.3%) statements pertaining to systemic treatment in adult and pediatric AA, respectively. @*Conclusion@#The present study produced up-to-date, evidence-based treatment guidelines for AA associated with the consensus obtained by experts based on the Korean healthcare system.
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Background@#Eosinophilic cellulitis, also known as Wells’ syndrome, is an uncommon skin disease characterized by recurrent pruritic polymorphous plaques or nodules. There is limited knowledge in the Korean literature regarding the clinical characteristics and course of eosinophilic cellulitis. @*Objective@#To investigate the clinical and histological features of eosinophilic cellulitis in Korean patients. @*Methods@#In this case series study, we retrospectively reviewed the electronic medical records of patients diagnosed with eosinophilic cellulitis at our institutions from 2004 to 2022. We also analyzed the literature published in dermatology and internal medicine journals in Korea. @*Results@#A total of 17 patients were included in this study (age, 39.4±21.8 years; 12 male [70.6%]), with an average disease duration of 4.4 months. We identified six clinical variants, with the typical plaque type being the most common (33.3%), followed by the papulonodular type (22.2%). Eosinophilia was observed in 78.6% of patients, and a flame figure, which is a typical histopathological feature of eosinophilic cellulitis, was observed in 52.9% of patients. Systemic and topical steroids and antihistamines were used as first-line treatment with generally favorable results (82.4%), followed by antibiotics (35.3%), cyclosporine (23.5%), and topical calcineurin inhibitors (23.5%).The recurrence rate during the mean follow-up period of 21.8 months was 29.4%. @*Conclusion@#Eosinophilic cellulitis can present with various clinical manifestations, and flame figures do not appear in all cases; therefore, clinical and histological correlations are critical for accurate diagnosis of patients with eosinophilic cellulitis.
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Increasing importance of vaccination during coronavirus disease 2019 (COVID-19) pandemic, several vaccines were developed. Messenger RNA (mRNA) vaccines including BNT162b2 (Pfizer, New York, NY, USA; BioNTech, Mainz, Germany), mRNA-1273 (Moderna, Cambridge, MA, USA) have been disclosed side effects such as thrombocytopenia, myocarditis and headache in general. In addition, adverse effects due to autoimmune responses induced by spike glycoproteins of mRNA vaccines are rarely reported such as the Guillain-Barre syndrome, autoimmune encephalitis and so on. Herein, we report a rare case of encephalitis with new-onset refractory status epilepticus after BNT162b2 mRNA COVID-19 vaccination improved with immune therapy.
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Background@#Alcohol consumption has been considered as a modifiable risk factor for dementia development and alcohol-related brain damage may further impair cognitive abilities in dementia patients. This study aimed to find out the differences in cognitive function according to current alcohol drinking in patients with self-perceived memory decline, including subjective cognitive decline (SCD), mild cognitive impairment (MCI) and early Alzheimer-type dementia (ATD). @*Methods@#From May 2018 to December 2019, retrospective chart review was performed in patients who visited CHA Bundang Medical Center for cognitive decline. A two-way analysis of variance with interaction test were used to analyze the impact of alcohol consumption on cognitive function between groups. @*Results@#A total of 147 patients was classified into three groups of SCD (n=30), MCI (n=53), and ATD (n=64), and each group was divided into two subgroups of alcohol users and alcohol non-users, according to the current status of alcohol consumption. Between SCD, MCI and ATD groups, scores of clock drawing test and Go/No-go test were significantly lower in current alcohol users of ATD groups compared to the SCD and MCI groups (p<0.05). @*Conclusions@#These results suggest that current alcohol consumption has detrimental effects especially on the frontal/executive function in early ATD patients. Considering the association between frontal/executive function and ADL, our finding suggests that cessation of alcohol intake may be a therapeutic strategy to prevent ADL deterioration in patients with ATD.
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Sleep-related head jerk (SRHJ) is conceived as a physiological motor phenomenon, which is mostly seen during rapid eye movement (REM) sleep. It should be distinguished from movements during REM sleep, mainly REM sleep behavior disorder (RBD). A 25-year-old male complained of daytime sleepiness with snoring and dream enactment behavior (DEB). Video polysomnography (PSG) excluded RBD and obstructive sleep apnea; frequent SRHJs during REM sleep with or without respiratory effort-related arousals, and SRHJs followed by one episode of DEB was observed. On PSG with continuous positive airway pressure titration, SRHJ with arousal events still remained but DEB symptom was resolved. REM without atonia unrelated to head jerks was not observed on all PSG data. Excessive SRHJs with arousal related to a sleep disturbance could indicate an SRHJ disorder, which might be a novel sleep-related movement disorder. In addition, clinicians should be careful in diagnosing young patients with RBD.
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Objectives@#Cognitive behavioral therapy for insomnia (CBT-I) is the first line treatment for insomnia. However, many patients remain with sleep disturbances even after undergoing CBT-I, and those with short sleep durations have shown fewer gains. Acceptance and commitment therapy (ACT) is one of the third wave of behavioral therapies, and it is useful in helping patients get flexibility of mind. Therefore, we incorporated its components into CBT-I, came up with an advanced CBT-I program that involves cognitive behavior therapy based on ACT, and examined its efficacy in comparison to that of CBT-I. @*Methods@#Patients with chronic primary insomnia were recruited at the memory center of CHA University Hospital from June to August 2020. To examine the efficacy of advanced CBT-I compared to that of CBT-I, the patients (n=16) were assigned to two groups (CBT-I: n=6; advanced CBT-I: n=10). The patients in each group were treated for 4 weeks (8 sessions). The quality of sleep, severity of insomnia, sleepiness, depression, anxiety, acceptance, efforts to sleep, and dysfunctional beliefs concerning sleep were assessed with self-report questionnaires. @*Results@#The severity of insomnia, quality of sleep, depression, anxiety, acceptance, efforts to sleep, and dysfunctional beliefs concerning sleep improved after both CBT-I and advanced CBT-I treatment. @*Conclusions@#This study examined the efficacy of advanced CBT-I in improving the severity of insomnia, sleep quality, and other symptoms related to sleep. The results suggest that components of ACT were useful for insomnia.
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Objectives@#Cognitive behavioral therapy for insomnia (CBT-I) is the first line treatment for insomnia. However, many patients remain with sleep disturbances even after undergoing CBT-I, and those with short sleep durations have shown fewer gains. Acceptance and commitment therapy (ACT) is one of the third wave of behavioral therapies, and it is useful in helping patients get flexibility of mind. Therefore, we incorporated its components into CBT-I, came up with an advanced CBT-I program that involves cognitive behavior therapy based on ACT, and examined its efficacy in comparison to that of CBT-I. @*Methods@#Patients with chronic primary insomnia were recruited at the memory center of CHA University Hospital from June to August 2020. To examine the efficacy of advanced CBT-I compared to that of CBT-I, the patients (n=16) were assigned to two groups (CBT-I: n=6; advanced CBT-I: n=10). The patients in each group were treated for 4 weeks (8 sessions). The quality of sleep, severity of insomnia, sleepiness, depression, anxiety, acceptance, efforts to sleep, and dysfunctional beliefs concerning sleep were assessed with self-report questionnaires. @*Results@#The severity of insomnia, quality of sleep, depression, anxiety, acceptance, efforts to sleep, and dysfunctional beliefs concerning sleep improved after both CBT-I and advanced CBT-I treatment. @*Conclusions@#This study examined the efficacy of advanced CBT-I in improving the severity of insomnia, sleep quality, and other symptoms related to sleep. The results suggest that components of ACT were useful for insomnia.
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Background@#Understanding the age-related morphological changes of facial soft tissue is fundamental in achieving improved outcomes of rejuvenating procedures. Three-dimensional (3D) photogrammetry is a reliable and convenient anthropometric tool to assess facial soft tissue. @*Objective@#The aim of this study was to establish age-related facial soft tissue morphology in Korean adult females using non-invasive 3D photogrammetry. @*Methods@#One hundred and ninety-two female participants were divided into three groups based on age: the younger group (aged 20∼39 years), middle group (40∼59 years), and older group (60∼79 years). Thirty-six landmarks were identified via 3D photogrammetric scanning (Morpheus 3D, Morpheus Co., Ltd., Seongnam, Korea). Fortyone facial dimensions were analyzed using the imaging software to find significance between the age groups. @*Results@#Smaller upper-facial volume (p=0.019) and shorter upperfacial height (p=0.034) were observed in the older group than in the younger group. In the mid-face, narrowed palpebral fissure (p＜0.001) with elongated upper eyelid height (p＜0.001) and widened nose (p＜0.001) were observed in the older group compared with the younger group. Longer lower-facial height (p＜0.001) with longer and wider philtrum (p＜0.001, p=0.004, respectively), shorter lower vermilion height (p＜0.001), wider mouth width (p＜0.001), and smaller lower vermilion angle (p＜0.001) were seen in the older group when compared with the younger group. Moreover, greater angles of nasofrontal, nasomental, and labiomental angle (p=0.015, p=0.015, p=0.080, respectively), and smaller nasofacial angle (p=0.034) were observed in the older group than in the younger group. @*Conclusion@#Our results provide clues of aging-related facial morphological characteristics in Korean female population.
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Jumping stump syndrome is considered to be a peripherally induced movement disorder due to damage to peripheral nerves leading to dystonia or myoclonus. Anti-leucine-rich glioma-inactivated 1 antibody (anti-LGI 1 Ab) encephalitis is clinically characterized with progressive cognitive dysfunction and seizure including facial brachial dystonic seizure. We report a case of a woman with a history of intractable involuntary movement on amputated forearm diagnosed as anti-LGI 1 Ab encephalitis, mimicking symptoms of jumping stump syndrome.
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@#Objectives: We aimed to investigate the demographics and medical management factors associated with dependence on hypnotics among outpatients with neurological disorders and insomnia. Methods: We reviewed electronic medical records of patients who received an initial hypnotic prescription between January 2014 and January 2016 and had later visited a neurological outpatient clinic before January 2018. We assessed patient demographics, the effectiveness of hypnotics, prescription periods, and hypnotic intake methods during the follow-up period. Results: Of 242 patients diagnosed with insomnia, we enrolled 114 patients (more women than men, at 61.4 versus 38.6%) who visited outpatient clinics regularly during the follow-up period. The mean age at onset was 65.8 ± 14.4 years. The most frequent neurological disorder was cerebrovascular disease, followed by neurodegenerative disease. During the 2-year period, 35.9% of participants remained hypnotics-free. Patients on zolpidem showed significantly greater insomnia improvement with hypnotic discontinuation than those on benzodiazepines and combination therapy (p=0.004). However, the type of hypnotics and demographic factors were not found to be independent risk factors. Multivariable analysis showed that longer periods between regular visits and a lower ratio of receiving number of pills to the time interval (days) between regular visits were independent risk factors for dependence on hypnotics. Conclusions: We found that low-dose and/or intermittent intake of hypnotics as well as frequent doctor visits could prevent dependence on hypnotics. It is important to establish the best practical guidelines for medical hypnotics management in outpatient primary care settings, including neurological clinics.
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Desmoplastic fibroblastoma is a rare fibrous tumor that usually presents as a painless, slow-growing mass in the subcutaneous tissues and skeletal muscles. It has a wide anatomic distribution, with the most common involvement being the arm and shoulder. Here, we report a case of a tiny painful desmoplastic fibroblastoma arising on the scalp. According to a microscopic examination, this tumor was composed of spindle-shaped fibroblasts in the dense collagenous stroma. On immunohistochemical staining, tumor cells were positive for vimentin and negative for smooth muscle actin, CD34, and S100. Our case is unique in that desmoplastic fibroblastoma developed on the scalp and there was presence of pain despite its small size.
Subject(s)
Actins , Arm , Collagen , Fibroblasts , Muscle, Skeletal , Muscle, Smooth , Scalp , Shoulder , Subcutaneous Tissue , VimentinABSTRACT
Radix Polygalae (RP) has been used to relieve psychological stress in traditional oriental medicine. Recently, cell protective, antiamnestic and antidepressant-like effects were disclosed but the possible application of RP to post-traumatic stress disorder, in which exaggerated fear memory persists, has not yet been explored. For this purpose, the effects of RP on fear behavior was examined in a mouse model of single prolonged stress and conditioned fear (SPS-CF), previously shown to mimic key symptoms of post-traumatic stress disorder. Male mice received daily oral dose of RP extract or vehicle during the SPS-CF procedure. Then fear-related memory (cohort 1, n=25), non-fear-related memory (cohort 2, n=38) and concentration-dependent effects of RP on fear memory (cohort 3, n=41) were measured in 3 separate cohort of animals. Also working memory and anxiety-like behaviors were measured in cohort 1. RP-treated SPS-CF mice exhibited attenuated contextual but not cued freezing and no impairments in the working memory and spatial reference memory performances relative to vehicle-treated SPS-CF controls. RP-treated SPS-CF and naive mice also demonstrated no difference in anxiety-like behavior levels relative to vehicle-treated SPS-CF and naive controls, respectively. In the hippocampus of SPS-CF mice, expression of BAG1, which regulates the activity of GR, was decreased, whereas RP increased expression of BAG1 in naïve and SPS-CF mice. These results suggest that RP exerts some symptomatic relief in a mouse with exaggerated fear response. RP and its molecular components may thus constitute valuable research targets in the development of novel therapeutics for stress-related psychological disorders.
Subject(s)
Animals , Humans , Male , Mice , Anxiety , Cohort Studies , Freezing , Hippocampus , Medicine, East Asian Traditional , Memory , Memory, Short-Term , Polygala , Stress Disorders, Post-Traumatic , Stress Disorders, Traumatic , Stress, PsychologicalABSTRACT
To identify the interchangeability of V(H) and V(L) framework region (FR) residues, we artificially introduced random mutations at all residue positions in a chicken monoclonal antibody, which has only one functional V(H) and Vλ gene. When we classified the amino acids into 5 groups by their physicochemical properties, all FR residues could be replaced by another group except L23 (C), H36 (W), H86 (D), H104 (G), and H106 (G). Eighty-two (50.9%), 48 (29.8%), 17 (10.6%), and 9 FR residues (5.6%) could be replaced by 4, 3, 2, and 1 group(s), individually, without significant loss of reactivity. We also confirmed a similar level of versatility with 2 different chicken antibodies. This high level of versatility on FR residues has not been predicted because it has not been observed in the 150 chicken antibodies that we previously generated or in the 1,269 naïve chicken V(H) sequences publically available. In conclusion, chicken antibody FR residues are highly interchangeable and this property can be applied for improving the physicochemical property of antibody including thermal stability, solubility and viscosity.
Subject(s)
Amino Acids , Antibodies , Chickens , Immunoglobulin Variable Region , Solubility , Somatic Hypermutation, Immunoglobulin , ViscosityABSTRACT
BACKGROUND: Kaempferol (3,4′,5,7-tetrahydroxyflavone) is a flavonoid known to have a wide range of pharmacological activities. The 3-OH group in flavonoids has been reported to determine antioxidant activities. OBJECTIVE: We tested whether kaempferol can affect the expression of integrins and the stem cell fate of interfollicular epidermal stem cells. METHODS: Skin equivalent (SE) models were constructed, and the expression levels of stem cell markers and basement membrane-related antigens were tested. The immunohistochemical staining patterns of integrins, p63, and proliferating cell nuclear antigen (PCNA) were compared between kaempferol- and apigenin-treated SE models. Reverse transcription-polymerase chain reaction (RT-PCR) was used to evaluate the mRNA expression of integrins. RESULTS: Kaempferol increased the thickness of the epidermis when added to prepare SEs. In addition, the basal cells of kaempferol- treated SEs appeared more columnar. In the immunohistological study, the expression of integrins α6 and β1 and the numbers of p63- and PCNA-positive cells were markedly higher in the kaempferol-treated model. However, apigenin showed no effects on the formation of three-dimensional skin models. RT-PCR analysis also confirmed that kaempferol increased the expression of integrin α6 and integrin β1. CONCLUSION: Our findings indicated that kaempferol can increase the proliferative potential of basal epidermal cells by modulating the basement membrane. In other words, kaempferol can affect the fate of interfollicular epidermal stem cells by increasing the expression of both integrins α6 and β1. These effects, in particular, might be ascribed to the 3-OH group of kaempferol.