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Neurothekeoma is a rare, benign, cutaneous tumor of unknown origin that can be classified into myxoid, cellular, and mixed types. The tumor presents as a solitary nodule mainly on the head, neck, and upper extremities of children and young adults with female preponderance. Histopathologically, epithelioid to spindled-shaped cells form lobules or micronodules and cytologic atypia are occasionally observed with variable fibrous and myxoid stroma.Neurothekeoma can mimic several benign and malignant tumors and recur if incompletely excised. Therefore, it is important to be aware of this tumor for accurate diagnosis. Herein, we report a case of nasal cellular neurothekeoma with a review of related literature.
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Background@#Many aspects are considered when selecting psoriasis treatments, such as psoriasis severity. In particular, patients’ subjective aspects, such as quality of life and treatment satisfaction, are important when planning treatment, and several studies on these aspects exist. However, studies on the direct thoughts of Korean patients with psoriasis, such as what patients value the most, are limited. @*Objective@#This study aimed to evaluate treatment satisfaction and perceptions regarding the importance or subjective achievement of goals in Korean patients with psoriasis treated with biologics or oral agents. @*Methods@#A total of 59 patients with psoriasis treated with biologics or oral agents for ＞16 weeks completed the questionnaire. The questionnaire consisted of four main parts: patient information, past history and treatment experience associated with psoriasis, treatment satisfaction, and subjective importance and degree of achievement of treatment goals in daily life. @*Results@#Treatment satisfaction in the biologics group was higher in terms of effectiveness, improvement in symptoms, speed of improvement, method of administration, frequency of administration, and comprehensive aspects than that in the oral agents group (p＜0.05). However, in both groups, the degree of improvement did not reach the patients’ subjective importance, and “concerned with other people’s eyes due to lesions in exposed area” had the biggest difference. @*Conclusion@#Although biologics had higher treatment satisfaction than oral agents, in some parts of daily life, the degree of improvement did not reach the patients’ subjective importance; therefore, some points still needs to be improved.
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Generalized pustular psoriasis of pregnancy (GPPP), characterized by widespread sterile pustules and erythematous patches with systemic symptoms such as fever, is a rare form of pustular psoriasis. GPPP typically occurs in the third trimester of pregnancy and can be triggered by various factors such as infections, hypocalcemia, and drugs including N-butyl-scopolammonium bromide. We report a rare case of new-onset GPPP in a 33-yearold multigravida female at 17 weeks’ gestation, which occurred earlier than usual, after taking hydroxychloroquine for 3 weeks to treat systemic lupus erythematosus. She stopped her medications and was treated with systemic corticosteroid, but without improvement.Her medication was changed to systemic cyclosporine; her skin lesions improved, which completely resolved after delivery. This is the first case of GPPP developed following hydroxychloroquine use for systemic lupus erythematosus, which occurred earlier than usual and completely resolved after delivery. This case demonstrates that hydroxychloroquine can induce GPPP before the third trimester of pregnancy.
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Cutaneous and subcutaneous metastases from the neoplasm of visceral organs are uncommon and have been estimated to occur in only 0.7%∼9% of patients. Among these cases, breast cancer is the most frequently observed malignancy. The incidence of cutaneous metastasis of breast cancer is 23.9%. The most common clinical manifestation of cutaneous metastasis of breast cancer is non-fixed painless erythematous nodules on the anterior chest. However, cutaneous metastasis of breast cancer shows a wide range of clinical manifestations and can mimic benign dermatologic lesions such as erythema annulare centrifugum, contact dermatitis, cellulitis, and erysipelas. We report a 52-year-old woman who presented with diffuse purpuric macules and patches on the right trunk. Finally, based on the clinical and pathologic findings, a diagnosis of cutaneous metastasis of breast cancer was made, and the patient expired 1 year later.
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A 3-month-old male infant was transferred to our hospital due to bicytopenia. His bone marrow biopsy showed irregular bony trabeculae with cartilaginous core, which was consistent with osteopetrosis. In the genetic test, c.242del (p.Pro81Argfs*85) in TCIRG1 was found to be homozygotic, thus he was diagnosed with malignant infantile osteopetrosis. At 6 months of age, he received double-unit umbilical cord blood transplantation (UCBT) with the conditioning regimen including busulfan, cyclophosphamide, and rabbit anti-thymocyte globulin. Initially, single UCB was infused to the patient, but the post infusion viability of the UCB was unexpectedly low. Thus, another UCB was additionally infused. Cyclosporine and mycophenolate mofetil were used for graft-versus-host disease (GVHD) prophylaxis. Neutrophils and platelets were engrafted on day +13 and +33, respectively. With engraftment, he showed overall grade 4 acute GVHD involving the skin and gut, which was refractory to corticosteroids. Despite treating with low-dose weekly methotrexate (10 mg/m2) and oral beclomethasone, his symptoms persisted. After treating with ruxolitinib 2.5 mg/day for 2 weeks, and 5 mg/day thereafter, his diarrhea stopped in 2 weeks and his skin symptoms gradually improved over 3 months. The short tandem repeats showed 100% donor chimerism at 1 and 3 months after UCBT. Currently, 4 months after UCBT, he is 10 months old. The oral prednisolone has been tapered to 0.6 mg/kg/day, and the dose of ruxolitinib was decreased to 2.5 mg/day without recurrence of GVHD. We plan to taper off the immunosuppressive agents if his GVHD symptoms do not recur.
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Background@#Only a few studies have tried to assess factors relevant to the satisfaction of the participants in atopic dermatitis (AD) educational programs. More systematic modeling of this issue is needed. @*Objective@#To examine the benefit of a conjoint educational program for AD on patients and caregivers in a clinical setting. @*Methods@#In a half-day educational program called “AD school”, 831 people (493 patients and 338 family members) participated for 8 years. Various educational and entertaining programs were provided. The on-site survey was administered to measure participants’ satisfaction and perception of the benefit. We applied structural equation modeling to identify the relations among satisfaction and perception. @*Results@#A total of 209 family survey data was obtained and analyzed. The survey items were grouped into four categories. The categories were classified as individual education, group education, fun activity, and overall satisfaction (fun, benefit, intention to re-join and recommend to others). According to the model that we built, comprehensive group education was demonstrated to be the most relevant factor affecting overall satisfaction. @*Conclusion@#Our holistic approach would allow dermatologists to improve the efficacy of the conjoint educational program for AD.
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The occurrence of malignant tumor in proximity to an arthroplasty prosthesis has been a matter of debate since it was first reported in 1978. Upon considering the number of orthopedic implants used, the occurrence of malignancy is rare. Especially in case of angiosarcoma, only a few cases have been reported worldwide. In this case, we report an extremely rare case of angiosarcoma arising at the site of a revision total hip arthroplasty. A 69-year-old female had received total hip replacement on her left hip due to osteoarthritis 8 months ago. Four months later, she complained pain on her operated area, X-ray showed loosening of implanted cup on her left hip. Thereafter, erythematous and purpuric papules and nodules were developed and spread around on her left hip. Through the skin biopsy she was diagnosed with angiosarcoma, and then she died of a sharp deterioration. Herein, we report a rare case of angiosarcoma occurred after total hip replacement with a review of the literature.
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Background@#Only a few studies have tried to assess factors relevant to the satisfaction of the participants in atopic dermatitis (AD) educational programs. More systematic modeling of this issue is needed. @*Objective@#To examine the benefit of a conjoint educational program for AD on patients and caregivers in a clinical setting. @*Methods@#In a half-day educational program called “AD school”, 831 people (493 patients and 338 family members) participated for 8 years. Various educational and entertaining programs were provided. The on-site survey was administered to measure participants’ satisfaction and perception of the benefit. We applied structural equation modeling to identify the relations among satisfaction and perception. @*Results@#A total of 209 family survey data was obtained and analyzed. The survey items were grouped into four categories. The categories were classified as individual education, group education, fun activity, and overall satisfaction (fun, benefit, intention to re-join and recommend to others). According to the model that we built, comprehensive group education was demonstrated to be the most relevant factor affecting overall satisfaction. @*Conclusion@#Our holistic approach would allow dermatologists to improve the efficacy of the conjoint educational program for AD.
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The occurrence of malignant tumor in proximity to an arthroplasty prosthesis has been a matter of debate since it was first reported in 1978. Upon considering the number of orthopedic implants used, the occurrence of malignancy is rare. Especially in case of angiosarcoma, only a few cases have been reported worldwide. In this case, we report an extremely rare case of angiosarcoma arising at the site of a revision total hip arthroplasty. A 69-year-old female had received total hip replacement on her left hip due to osteoarthritis 8 months ago. Four months later, she complained pain on her operated area, X-ray showed loosening of implanted cup on her left hip. Thereafter, erythematous and purpuric papules and nodules were developed and spread around on her left hip. Through the skin biopsy she was diagnosed with angiosarcoma, and then she died of a sharp deterioration. Herein, we report a rare case of angiosarcoma occurred after total hip replacement with a review of the literature.
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Background@#Acanthosis nigricans is characterized by a velvety thickening of the epidermis accompanied by different degrees of hyperpigmentation, and known to be linked to obesity and insulin resistance. @*Objective@#We aimed to analyze obesity-related factors in acanthosis nigricans patients and to evaluate the correlations between acanthosis nigricans and various factors. @*Methods@#From January 2004 to February 2015, 27 acanthosis nigricans patients participated in this study. Blood samples were collected from a control group of seven overweight people and from the seven acanthosis nigricans patients, and they were analyzed for different obesity-related factors. Skin samples were collected from the 23 acanthosis nigricans patients and from 11 patients with epidermal nevi, and immunohistochemistry was performed to detect the presence of adiponectin receptor 1, adiponectin receptor 2, and the leptin receptor. @*Results@#The median serum leptin level in the acanthosis nigricans patients (13 ng/mL) was significantly higher than that in the overweight control individuals (8.9 ng/mL) (p=0.021). The acanthosis nigricans patients had significantly higher levels of insulin-like growth factor-1 in their serum samples (p=0.017). The immunohistochemical analysis determined that the skin from the acanthosis nigricans patients stained significantly more intensely for the leptin receptor compared with that seen in the skin from the patients with epidermal nevi (p=0.002). @*Conclusion@#In conclusion, this study’s findings suggest that the levels of leptin and insulin-like growth factor-1 in the serum, and the expression of the leptin receptor in the skin are elevated with acanthosis nigricans.
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Background@#Aquaporin 1 (AQP1) is a transmembrane channel protein that allows rapid transposition of water and gases, in recent discoveries of AQP1 function involve cell proliferation, differentiation, wound healing, inflammation and infection in different cell types, suggesting that AQP1 plays key roles in diverse biologic process. Until now, less is known about the function of AQP1 on ultraviolet radiation induced photoaged skin. @*Objective@#In this study we set out to examine whether AQP1 expression may be influenced by repeated irradiation of ultraviolet B (UVB) in cultured dermal fibroblasts. @*Methods@#To elucidate the function of AQP1 in skin photoaging, human dermal fibroblasts (HS68) were irradiated by a series of 4 sub-cytotoxic doses of UVB which are known as UV-induced cell premature senescence model. Reverse transcription polymerase chain reaction and Western blotting were conducted to detect AQP1 expression from different groups. Then, cells were transfected with AQP1-targeting small interfering RNA. The activities of signaling proteins upon UVB irradiation were investigated to determine which pathways are involved in AQP1 expression. @*Results@#AQP1 expression was increased by 100 mJ/cm2 of UVB irradiation, but decreased by 200 mJ/cm2. Depletion of the AQP1 increased the apoptotic sensitivity of cells to UVB, as judged by upregulation of the p53, p21, poly (adenosine diphosphate [ADP]-ribose) polymerase and Bax together with the increased Bax/Bcl2 ratio. UVB induced downregulation of AQP1 was significantly attenuated by pretreatment with the MEK/ERK inhibitor (PD98059). @*Conclusion@#We concluded that AQP1 expression was down-regulated by repeated exposure of UVB via MEK/ERK activation pathways. The AQP1 reduction by UVB lead to changes of physiological functions in dermal fibroblasts, which might be associated with the occurrence and development of UVB induced photoaging.
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Severe skin reactions complicating tumor necrosis factor-alpha (TNF-α) antagonist therapy were somewhat reported in adults. We report a case of 24-month-old boy with refractory Kawasaki disease who developed toxic epidermal necrolysis (TEN) after one dose of 5mg/kg infliximab. The patient responded to intravenous hydrocortisone and was able to discharge without any other serious complications. The clinicians should be aware of the potential for severe skin reactions from the use of TNF-α antagonists in children.
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Severe skin reactions complicating tumor necrosis factor-alpha (TNF-α) antagonist therapy were somewhat reported in adults. We report a case of 24-month-old boy with refractory Kawasaki disease who developed toxic epidermal necrolysis (TEN) after one dose of 5mg/kg infliximab. The patient responded to intravenous hydrocortisone and was able to discharge without any other serious complications. The clinicians should be aware of the potential for severe skin reactions from the use of TNF-α antagonists in children.
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No abstract available.
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Colágeno , Fibroblastos , Expressão Gênica , Proteínas Quinases p38 Ativadas por MitógenoRESUMO
Diffuse plane xanthoma (DPX) presents with symmetric yellow-orange plaques primarily on the neck, upper trunk, flexural folds, and the periorbital region. Based on serum lipid and lipoprotein levels, these xanthomas are classified as normolipemic or hyperlipoproteinemic DPX. Diffuse normolipemic plane xanthoma (DNPX) is a rare condition that is not well studied yet. It is associated with reticulo-endothelial diseases, particularly multiple myeloma and monoclonal gammopathy of unknown significance (MGUS). A 62-year-old woman developed yellowish hyperpigmented papules and diffuse patches in the medial canthal area of her neck. Based on a skin biopsy and laboratory analyses, she was diagnosed with DNPX associated with multiple myeloma. This diagnosis demonstrates that dermatological lesions should be carefully assessed as they may be the first manifestation of an underlying hematological disease. We report herein a rare case of diffuse plane xanthoma associated with multiple myeloma and review the relevant literature.
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Feminino , Humanos , Pessoa de Meia-Idade , Biópsia , Diagnóstico , Doenças Hematológicas , Lipoproteínas , Mieloma Múltiplo , Pescoço , Paraproteinemias , Pele , XantomatoseRESUMO
Azathioprine is an immunosuppressive drug that has been widely used in dermatology for the treatment of immunobullous diseases. Myelosuppression is the most important side effect and requires close observation of the complete blood cell count. The clinical findings of myelosuppression include general weakness, poor oral intake, nausea, dyspnea, and pallor. It can occur within several weeks to years after initial azathioprine treatment; thus, a weekly full blood count for the first 4 weeks, followed by reduced frequency of monitoring to a minimum of once every 3 months is recommended. If the myelosuppression is not treated properly, it can lead to fever, secondary infection, sepsis, and even death. Herein, we present three educational cases for dermatologists to order to underline the risk of myelosuppression during azathioprine treatment.
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Azatioprina , Contagem de Células Sanguíneas , Coinfecção , Dermatologia , Dispneia , Febre , Náusea , Palidez , SepseRESUMO
Terra firma-forme dermatosis is characterized by asymptomatic dirt-like papules and plaques. It appears as dirt-like lesions, but is known to develop in persons with normal hygiene habits. Terra firma-forme dermatosis is rarely reported and has an unknown etiology. It is hypothesized to occur as a result of abnormal keratinization. Terra firma-forme dermatosis is usually detected in children and young adults. Its characteristic features include disappearance with alcohol swabbing. Thus, wiping with alcohol-soaked cotton is recommended before any other examination in cases where terra firma-forme dermatosis is suspected.