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Primary cutaneous extraskeletal Ewing sarcoma (EWS) is a primitive neuroectodermal tumor that usually occurs as a small, localized tumor on the trunk or extremities of young adults. The prognosis is typically reported to be quite favorable. It is extremely rare; only three cases of primary cutaneous EWS have been reported in Korea. In the first report, molecular genetic testing was not performed to make a definitive diagnosis. In the second report, reverse transcription polymerase chain reaction (RT-PCR) for EWS-FLI1 gene arrangement was done, but the result was negative. Although RT-PCR and fluorescence in situ hybridization (FISH) were performed in the third report, none of the results were shown in the article. Considering that genetic testing is an essential diagnostic tool for certain diseases, such as some brain tumors, we report a case of primary cutaneous extraskeletal EWS, including the result of RT-PCR. A 36-year-old Korean female presented with a cutaneous mass on the abdomen. Histological evaluation revealed solid sheets of primitive, small, uniform cells with hyperchromatic nuclei and scant cytoplasm. Immunohistochemistry stains were positive for CD99 and FLI1. RT-PCR showed a t(11;22) EWSR1 (Ewing sarcoma region 1)-FLI1 (Friend leukemia virus integration 1) translocation.
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Background@#Pediatric alopecia areata (AA) can affect the quality of life (QoL) of patients and their family members. Research on the QoL and burden on family members in pediatric AA is limited. @*Objective@#This nationwide multicenter questionnaire study described the QoL and burden of the family members of patients with pediatric AA. @*Methods@#This nationwide multicenter questionnaire study enrolled AA patients between the ages of 5 and 18 years from March 1, 2017 to February 28, 2018. Enrolled patients and their parents completed the modified Children’s Dermatology Life Quality Index (CDLQI) and the modified Dermatitis Family Impact (mDFI). The disease severity was measured using the Severity of Alopecia Tool (SALT) survey scores. @*Results@#A total of 268 patients with AA from 22 hospitals participated in this study. Our study found that the efficacy and satisfaction of previous treatments of AA decreased as the severity of the disease increased. The use of home-based therapies and traditional medicines increased with the increasing severity of the disease, but the efficacy felt by patients was limited. CDLQI and mDFI scores were higher in patients with extensive AA than those with mild to moderate AA. The economic and time burden of the family members also increased as the severity of the disease increased. @*Conclusion@#The severity of the AA is indirectly proportional to the QoL of patients and their family members and directly proportional to the burden. Physicians need to understand these characteristics of pediatric AA and provide appropriate intervention to patients and their family members.
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Secukinumab is an anti-interleukin 17A monoclonal antibody used for treating chronic plaque psoriasis. Eczematous eruption is a relatively unknown adverse effect of secukinumab. A 32-year-old man developed eczematous eruptions on both lower extremities following secukinumab treatment for severe plaque psoriasis. The lesions were clinically diagnosed as a nummular eczema-like eruption and were treated with topical corticosteroids without switching or stopping secukinumab. Considering the increased use of secukinumab and other interleukin-17A inhibitors, dermatologists should be aware of the cutaneous side effects of the drug.
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Background@#Sweet syndrome is characterized by tender erythematous plaques and nodules with predominantly dermal neutrophilic infiltrates. Sweet syndrome is relatively rare in children and adolescents. To date, only a few cases have been reported in Korean literature. @*Objective@#The aim of this study was to investigate the clinical and histopathological features of Sweet syndrome in Korean children and adolescents. @*Methods@#A retrospective study was conducted on 15 pediatric patients (aged ＜18 years) who were diagnosed with Sweet syndrome between 1991 and 2019. We reviewed the clinical and histopathological features of Sweet syndrome. @*Results@#The age of the 15 patients ranged from 4 months to 17 years. Among the 15 patients with Sweet syndrome, nine patients were females and six patients were males. Most patients (80%) had lesions on the upper extremities. Fever and tenderness (60%) were the most commonly associated symptoms. Transient infections such as upper respiratory infection or gastroenteritis were the most common identifiable cause, observed in 40% of patients. Histopathologically, dermal neutrophilic infiltration was observed in all patients. All patients were treated with systemic corticosteroids and showed a good response, although 26.7% of the patients experienced symptom recurrence.During the follow-up period, there were no incidences of any complications or extracutaneous manifestations in the patients. @*Conclusion@#In contrast to previous reports of pediatric Sweet syndrome, female predominance was observed in this study. Transient infection was the most common factor. All patients responded well to systemic corticosteroid therapy without complications or extracutaneous manifestations during the follow-up period.
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Background@#Sweet syndrome is characterized by tender erythematous plaques and nodules with predominantly dermal neutrophilic infiltrates. Sweet syndrome is relatively rare in children and adolescents. To date, only a few cases have been reported in Korean literature. @*Objective@#The aim of this study was to investigate the clinical and histopathological features of Sweet syndrome in Korean children and adolescents. @*Methods@#A retrospective study was conducted on 15 pediatric patients (aged ＜18 years) who were diagnosed with Sweet syndrome between 1991 and 2019. We reviewed the clinical and histopathological features of Sweet syndrome. @*Results@#The age of the 15 patients ranged from 4 months to 17 years. Among the 15 patients with Sweet syndrome, nine patients were females and six patients were males. Most patients (80%) had lesions on the upper extremities. Fever and tenderness (60%) were the most commonly associated symptoms. Transient infections such as upper respiratory infection or gastroenteritis were the most common identifiable cause, observed in 40% of patients. Histopathologically, dermal neutrophilic infiltration was observed in all patients. All patients were treated with systemic corticosteroids and showed a good response, although 26.7% of the patients experienced symptom recurrence.During the follow-up period, there were no incidences of any complications or extracutaneous manifestations in the patients. @*Conclusion@#In contrast to previous reports of pediatric Sweet syndrome, female predominance was observed in this study. Transient infection was the most common factor. All patients responded well to systemic corticosteroid therapy without complications or extracutaneous manifestations during the follow-up period.
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Perforating granuloma annulare (PGA), a rare variant of granuloma annulare, is characterized by transepidermal elimination of altered collagen that clinically manifests an umbilicated papule with a central crust. It can be confused with papulonecrotic tuberculid (PNT) because of their similar appearance. Unlike PGA, PNT is usually related to tuberculosis infection with a typical histologic finding of wedge-shaped dermal necrosis. Here, we report the first Korean case of PGA mimicking PNT both clinically and histologically. A 43-year-old Korean woman presented with erythematous papules localized on the extensor surface of her limbs for one year. Some of these papules had a central umbilication or a crust. Regarding comorbidity, she had latent tuberculosis diagnosed with QuantiFERON®-TB Gold test about five months ago. She was on antituberculous medication. Initially, a diagnosis of papulonecrotic tuberculid accompanied by latent tuberculosis was considered. However, despite taking the antituberculous medication for five months, her skin lesions were not improved. Biopsy specimen from her arm lesion showed wedge-shaped area of necrosis in the dermis. Additionally, there were multiple focal mucin depositions and palisading granulomatous inflammation throughout the dermis. A diagnosis of PGA was made and she was treated with topical corticosteroid. After two weeks of applying topical corticosteroid, most of her skin lesions disappeared, leaving some hyperpigmented scars.
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Adulto , Feminino , Humanos , Braço , Biópsia , Cicatriz , Colágeno , Comorbidade , Derme , Diagnóstico , Extremidades , Granuloma Anular , Granuloma , Inflamação , Tuberculose Latente , Mucinas , Necrose , Pele , Tuberculose , Tuberculose CutâneaRESUMO
No abstract available.
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Hiperlipoproteinemia Tipo IV , Hiperlipoproteinemias , XantomatoseRESUMO
BACKGROUND: Longitudinal melanonychia (LM) can be challenging as it may be caused by a wide variety of benign and malignant conditions. However, there are scarce data on LM confirmed by skin biopsy examination in Korean patients. OBJECTIVE: We sought to evaluate the clinical features and histopathologic diagnosis of LM in Korean patients. METHODS: We reviewed the medical records and clinical photographs of patients presenting with LM confirmed by skin biopsy examination between June 2007 and June 2017. RESULTS: Among a total of 75 patients with LM confirmed by skin biopsy examination at our hospital over a period of 10 years, 11 patients (14.7%) had malignant lesions. Common features of malignant lesions were involvement of a single nail, color heterogeneity, Hutchinson's sign, and nail plate dystrophy or ulceration. Common features of benign lesions were a negative Hutchinson's sign and absence of nail plate dystrophy and ulceration. CONCLUSION: This study analyzed the clinical features and histopathologic diagnosis of LM in Korean patients. Our data may contribute to determining the management approach for patients with LM.
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Humanos , Biópsia , Diagnóstico , Prontuários Médicos , Características da População , Pele , ÚlceraRESUMO
No abstract available.
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Nevus lipomatosus superficialis is a rare type of benign hamartomatous skin lesion characterized by the presence of mature adipose tissues in the dermis; it usually manifests as a protruding papule or groups of papules or nodules that have smooth or wrinkled surfaces. We report a case of nevus lipomatosus superficialis without external manifestations in a 49-year-old woman.
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Feminino , Humanos , Pessoa de Meia-Idade , Derme , Nevo , PeleRESUMO
No abstract available.
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Bowen's disease usually manifests as a slowly enlarging erythematous scaly patch or plaque. An uncommon variant of Bowen's disease showing a verrucous appearance has been reported and a distinct variant with a prominent clear cell change on histopathology, in addition to a verrucous surface change, was also reported. We describe novel form of Bowen's disease having a cerebriform appearance and showing histopathologically a significant clear cell change and propose that the clinical term “lobulated Bowen's disease” would be compatible for the description of this unique clinical variant. From a histopathological point of view, the precise definition and etiopathogenesis of the clear cell change in Bowen's disease should be elucidated.