ABSTRACT
Blastic plasmacytoid dendritic cell neoplasm (BPDCN) is a rare and aggressive lymphoma with an overall incidence of 0.04 cases per 100,000 people. BPDCN is a hematopoietic clonal neoplasm that originates from plasmacytoid dendritic cell precursors. A 63-year-old man presented with multiple erythematous nodules over his whole body, including his face, trunk, and both upper and lower extremities that appeared 1 month ago. Skin biopsy showed diffuse dermal infiltration by monomorphic atypical lymphocytes with large, irregular nuclei and scant cytoplasms. Immunohistochemical staining was positive for CD4, CD56, and CD123. The karyotype test showed abnormalities in male chromosomes 47, XY, ＋8 [2]/46, and XY [25], and mutations in DNMT3A, TET2, SRSF2, and ATRX genes were identified in a next-generation sequencing (NGS)-based acute myeloid leukemia gene panel test. The patient was diagnosed with BPDCN and treated with a KALLA 1406 regimen; however, he died on the 17th day of treatment.
ABSTRACT
Bullous pemphigoid (BP) is a chronic, autoimmune blistering disease that has concerning morbidity and mortality rates. Recently, several studies have focused on eosinophils due to their significant role in the pathogenesis of BP, considering that they are ubiquitous in the serum, tissue, and blister fluids of patients with BP. With this context, precision therapy that targets mediators of eosinophil activity could be a possible novel therapeutic strategy.Interleukin (IL)-5 is crucial for B-cell maturation, which consequently results in immunoglobulin production, and promotes eosinophil differentiation, proliferation, and activation. To our best knowledge, reslizumab has not yet been reported to treat BP. Herein, we report a case of steroid- and omalizumab-resistant BP treated successfully using reslizumab. Our data suggest that IL-5 could be a novel specific biologic target within the entire immunopathogenesis of BP, and reslizumab would be a novel therapeutic modality.
ABSTRACT
Myxoinflammatory fibroblastic sarcoma (MIFS) is a rare neoplasm that is frequently located in the distal extremities. Emerging evidence suggests that MIFS can also affect the proximal limbs, trunk, and scalp, and aggressive clinical courses have been noted. We report a case of MIFS that occurred suddenly in the patient’s forearm and grew rapidly within 2 weeks. A level of Ki-67 was observed in the patient’s lesion, which constitutes a considerable finding compared with most MIFS cases. The patient underwent surgical tumor removal, and no evidence of recurrence was noted. We highlight this case in view of its sudden occurrence and rapid local progression, which contradicts the usual features of this disease, suggesting that this clinical course might be attributable to the high Ki-67 value.
ABSTRACT
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.
ABSTRACT
Alopecia areata is a chronic organ-specific autoimmune disease and it could be associated with other autoimmune diseases. We, herein, report a case of alopecia areata in a patient with a thymoma without myasthenia gravis. Multiple hairless patches rapidly developed 6 weeks before the first visit on the patient who had been newly diagnosed with thymoma 2 weeks before the hairless patches occurred, and thymectomy was done 2 weeks before visiting dermatologic department. She had no symptoms associated with myasthenia gravis, and there were no abnormal findings on neurologic exams and acetylcholine receptor autoantibody was not detected in serum. Scalp biopsy showed numerous lymphocytic inflammations around hair follicles and in immunohistochemical staining, the aggregation of CD4+ and CD8+ T cells was observed around hair follicles and FoxP3+ T lymphocytes were rarely observed around hair follicles. The patient refused any treatment and her hairless patches were completely recovered 3 months after thymectomy, without being recurred 3 years after thymectomy. On the basis of both clinical manifestations and histologic findings, we concluded that alopecia areata in the patient had developed in association with thymoma and was recovered rapidly after thymectomy.
ABSTRACT
Background@#Chronic actinic dermatitis (CAD) is an uncommon eczematous photosensitivity disorder having a well-known relationship with allergic or photoallergic contact dermatitis. Moreover, the prognosis of CAD is worse in patients with lower minimal erythema doses (MEDs). However, the correlations between disease duration, contact and photocontact allergens, and MED values in CAD have not been reported. @*Objective@#To investigate the correlations among MED values, disease duration, and allergic patch/photopatch testing results and to elucidate the correlation between clinical severity and contact/photocontact allergens. @*Methods@#This study included 121 patients with CAD. Clinical data were analyzed according to the MED values, disease durations, and contact/photocontact allergens. Phototesting and patch/photopatch testing was performed in all the patients. @*Results@#The MED value was inversely correlated with disease duration for both MED-ultraviolet A and MED-ultraviolet B, with lower MED values for higher numbers of positive items in the patch/photopatch test. The most frequently detected allergens in the patch and photopatch tests were p-phenylenediamine (n=36, 9.3%) and Balsam of Peru (n=29, 21.3%). Clinically, patients demonstrating a greater number of detected allergens show poorer clinical severities (p＜0.05). @*Conclusion@#The number of contact/photocontact allergens correlates with the severity of CAD.
ABSTRACT
A cherry hemangioma is a type of capillary hemangioma that commonly occurs in older adults. Despite its benign nature, treatment is often required for cosmetically unacceptable lesions, and therapeutic options include electrocoagulation, sclerotherapy, cryotherapy, or laser therapy. Laser therapy using a V-beam and long-pulsed neodymium-doped yttrium aluminum garnet laser has shown favorable outcomes in patients with hemangiomas. We report two cases of cherry hemangiomas treated with the ‘Pharaon LipoⓇ laser’, an advanced and powerful diode laser that is not commonly used for hemangiomas but is selected for lipolysis and varicose vein coagulation. A 64-year-old German man presented with a several-decade history of multiple erythematous maculopapules on his chest, and a 30-year-old Korean man presented with a one-year history of similar lesions on his abdomen. Histopathological evaluation of biopsy specimens confirmed diagnosis of cherry hemangiomas. We successfully treated these lesions using the Pharaon Lipo Ⓡlaser without any adverse effect.
ABSTRACT
Background@#Chronic actinic dermatitis (CAD) is an uncommon eczematous photosensitivity disorder having a well-known relationship with allergic or photoallergic contact dermatitis. Moreover, the prognosis of CAD is worse in patients with lower minimal erythema doses (MEDs). However, the correlations between disease duration, contact and photocontact allergens, and MED values in CAD have not been reported. @*Objective@#To investigate the correlations among MED values, disease duration, and allergic patch/photopatch testing results and to elucidate the correlation between clinical severity and contact/photocontact allergens. @*Methods@#This study included 121 patients with CAD. Clinical data were analyzed according to the MED values, disease durations, and contact/photocontact allergens. Phototesting and patch/photopatch testing was performed in all the patients. @*Results@#The MED value was inversely correlated with disease duration for both MED-ultraviolet A and MED-ultraviolet B, with lower MED values for higher numbers of positive items in the patch/photopatch test. The most frequently detected allergens in the patch and photopatch tests were p-phenylenediamine (n=36, 9.3%) and Balsam of Peru (n=29, 21.3%). Clinically, patients demonstrating a greater number of detected allergens show poorer clinical severities (p＜0.05). @*Conclusion@#The number of contact/photocontact allergens correlates with the severity of CAD.
ABSTRACT
A cherry hemangioma is a type of capillary hemangioma that commonly occurs in older adults. Despite its benign nature, treatment is often required for cosmetically unacceptable lesions, and therapeutic options include electrocoagulation, sclerotherapy, cryotherapy, or laser therapy. Laser therapy using a V-beam and long-pulsed neodymium-doped yttrium aluminum garnet laser has shown favorable outcomes in patients with hemangiomas. We report two cases of cherry hemangiomas treated with the ‘Pharaon LipoⓇ laser’, an advanced and powerful diode laser that is not commonly used for hemangiomas but is selected for lipolysis and varicose vein coagulation. A 64-year-old German man presented with a several-decade history of multiple erythematous maculopapules on his chest, and a 30-year-old Korean man presented with a one-year history of similar lesions on his abdomen. Histopathological evaluation of biopsy specimens confirmed diagnosis of cherry hemangiomas. We successfully treated these lesions using the Pharaon Lipo Ⓡlaser without any adverse effect.
ABSTRACT
Synchronous primary cancers in the liver and gallbladder have been rarely reported.We report a case of synchronous cancers of hepatic angiosarcoma and gallbladder adenocarcinoma, mimicking gallbladder cancer with hepatic invasion. Additionally, the clinical implications, the radiologic features, and the diagnostic difficulties are further discussed.
ABSTRACT
Background@#Alopecia areata (AA) is a T cell-mediated autoimmune disease. In patients with chronic AA, hair loss occurs because of the insidious destruction of the stem cells in the hair bulge and hair-bulb matrix along with the attack of the cytotoxic T cells and infiltration of the T-helper 17 cells. Cyclosporine (CsA) inhibits the action of calcineurin in hair stem cells and subsequently induces the inhibition of the nuclear factor of activated T cells c1 and expression of cyclin-dependent kinase 4, thereby stimulating hair regrowth.Objective The purpose of this study was to evaluate the long-term therapeutic outcomes of low-dose systemic CsA treatment in patients with chronic AA, relative to their initial severity of AA. @*Methods@#A total of 98 outpatients with chronic AA, who were treated with low-dose systemic CsA, were included in the study. @*Results@#Among the 98 patients, 72 (73.5%) had more than 50% hair regrowth, while 30 patients (30.6%) had more than 90% hair regrowth after 18 months of CsA treatment. Patients with a lower initial severity of alopecia tool (SALT) score demonstrated better treatment outcomes than those with a higher initial SALT score. @*Conclusion@#Patients with chronic AA who were treated with low-dose, systemic CsA experienced significant hair regrowth with fewer side effects. Therefore, the use of low-dose, systemic CsA is recommended in the treatment of patients with chronic AA.
ABSTRACT
Background@#Vitiligo is a skin depigmentation disorder, for which, repigmentation treatment with combined follicular unit extraction (FUE) graft and narrowband ultraviolet B (NBUVB) is considered superior to micro-punch graft therapy. BMP4 can induce MITF expression in Neural crest stem cells (NCSCs), and α-MSH subsequently promotes the differentiation of MITF-expressing cells along the melanocyte lineage. @*Objective@#To investigate why FUE grafting is superior to epidermal mini grafting in promoting hair follicles (HF) melanocyte cell survival and longevity, we planned the in vitro experiments HF bulge NCSCs differentiate into melanocyte precursors under the co-treatment of BMP4 and α-MSH. @*Methods@#Cells that migrated from the HF bulge of scalp were cultured and assessed using immunofluorescence. Transcriptome analysis was performed on RNA sequencing results. @*Results@#Basic fibroblast growth factor promotes the proliferation and survival of NCSCs, with spontaneous differentiation into SOX10+/SOX2+ glial progenitors, but not into SOX10+/MITF+ precursor melanocytes. Both BMP4 and α-MSH promoted the differentiation into MITF-expressing cells. RNA sequencing revealed a downregulation in neu-regulin-1 (NRG1) and sermaphorin 3C (SEMA3C), and upregulation in WNT10A. Furthermore, FUE grafting had a source of reservoir melanocytes superior to mini- grafting in treatment for vitiligo. @*Conclusion@#We obtained SOX10+/ MITF+ precursor melanocytes through an induction of differentiation along the melanocyte lineage by BMP4 and α -MSH. According to the RNA sequencing results that NRG1 and SEMA3C were downregulated and WNT10A was upregulated, we postulated that HF NCSCs differentiated into melanocyte by co-treatment of BMP4 and α-MSH. Overall, FUE grafting is a more robust and substitutive treatment option for vitiligo.
ABSTRACT
Skin disease can be caused by high temperature, and it is related to the temperature regulation mechanism of human body, adaptation reaction to temperature change, and health problems due to the recent problematic climate change. In hyperthermia, hot and dry skin is typical manifestation, and sometimes the skin color turns red. On the other hand, the skin color can become pale in severe febrile convulsion. Burn is a skin damage caused by heat, and not only the skin but also the underlying tissues can be destroyed in severe case. It is important to determine the degree and extent of the burn to treat adequately. In the case of severe burns, systemic treatment and prevention of infection or shock should be needed. Miliaria, also called “sweat rash,” occurs when the sweat is accumulated as the sweat gland is closed and sweat cannot be secreted to the surface of the skin. The basis of treating miliaria is to keep the patient in a cool environment. Erythema ab igne is defined as a network of hyperpigmentation that occurs after prolonged exposure to heat that is not enough to cause burn. It may disappear when exposure to heat is interrupted, but it may remain permanently. The extent and mechanism of heat-induced skin disease very diverse and it should be carefully assessed for the severity of each disease, the treatment method and prognosis.
Subject(s)
Humans , Burns , Climate Change , Erythema , Fever , Hand , Hot Temperature , Human Body , Hyperpigmentation , Methods , Miliaria , Prognosis , Seizures, Febrile , Shock , Skin Diseases , Skin Pigmentation , Skin , Sweat , Sweat GlandsABSTRACT
Skin diseases associated with athletic activities can be classified as skin infections, inflammatory reactions, trauma, and abnormal proliferation, depending on the cause of the condition. Athlete's nodule is a generic term for reactive nodules that occur in athletes. It is particularly common in the foot due to tight sneakers, repetitive pressure or friction, and inappropriate choice of shoes or other sports equipment. The diagnosis of black heel (calcaneal petechiae) should be considered when numerous black spots occur on the soles in patients who frequently engage in abrupt movements, such as starts, stops, or leaps. Palmoplantar eccrine hidradenitis may occur in athletes who play baseball, dance, and climb, activities in which repetitive and strong stimuli are applied to the floor of the hands and feet. Painful fat herniation should be suspected in cases of painful skin-colored firm nodules on the feet of athletes who place a large amount of weight on their feet when moving. Itching, urticaria, angioedema, chest tightness, and syncope occurring within 5 minutes after starting exercise should be suspected to be exercise-induced angioedema/anaphylaxis. Excessive force can cause deformation of nails, as in tennis toe and jogger's toenail. For the diagnosis and treatment of sports-related skin diseases, it is essential to pay attention to patients' hobbies and exercise habits, including sports, and to consider the relationship of those habits with the presumed mechanisms of the skin disease. In addition, thorough pre-exercise warm-ups, increasing strength gradually, and wearing proper equipment will help prevent the occurrence of sports-related skin diseases.
Subject(s)
Humans , Angioedema , Athletes , Baseball , Dancing , Diagnosis , Foot , Friction , Hand , Heel , Hidradenitis , Hobbies , Nails , Pruritus , Shoes , Skin Diseases , Skin , Sports , Sports Equipment , Syncope , Tennis , Thorax , Toes , UrticariaABSTRACT
OBJECTIVE: This study examined the clinical significance of microscopic hematuria and grade of hydronephrosis in ureteral calculi patients visiting the emergency department (ED). METHODS: The EDs of two medical centers were reviewed retrospectively from August 1, 2012 to July 31, 2017. The total ureteral calculi patients were 2,069 and 1,593 patients were analyzed after exclusion. The normal range of microscopic hematuria in both medical centers was 0–5/high power field (HPF) of red blood cell in urinalysis. Therefore, microscopic hematuria more than 5/HPF was defined as positive. The size of the stone, grade of hydronephrosis (normal, mild, moderate, and severe) and location of ureteral calculi in the non-contrast abdomen and pelvis computed tomography (CT) was measured by one person at each medical center using same method. The patients with or without microscopic hematuria and grade of hydronephrosis were then compared. RESULTS: The median size of the ureteral calculi patients without and with microscopic hematuria was 5.4 mm (range, 3.8–9.0 mm) and 4.0 mm (range, 3.0–5.8 mm) (P < 0.001). The patients grouped as normal to mild, and moderate to severe hydronephrosis, without microscopic hematuria was 191 (16.5%) and 91 (20.9%), respectively. On the other hand, microscopic hematuria was 966 (83.5%) and 345 (79.1%) (P=0.042). According to the location of ureteral calculi, with and without microscopic hematuria was 81.9% and 18.1% in the upper ureter, 82.9% and 17.1% in the mid ureter, and 82.6% and 17.4% in the lower ureter, respectively (P=0.935). CONCLUSION: The median size of the stone and grade of hydronephrosis were related to microscopic hematuria but the location of the ureteral calculi was not related. Therefore, in cases without microscopic hematuria in suspected ureteral calculi, clinicians should check the abdomen and pelvis CT for an accurate diagnosis and treatment of ureteral calculi.
Subject(s)
Humans , Abdomen , Diagnosis , Emergencies , Emergency Service, Hospital , Erythrocytes , Hand , Hematuria , Hydronephrosis , Methods , Pelvis , Reference Values , Retrospective Studies , Ureter , Ureteral Calculi , UrinalysisABSTRACT
Imiquimod, a toll-like receptor agonist, is a topical immunomodulator that induces the production of several cytokines including interferon-alpha, which shows antifibrotic properties. We hypothesized that the antifibrotic effect of imiquimod would soften fibrotic skin lesions. Therefore, we applied topical imiquimod with topical tacrolimus or systemic acitretin in patients with refractory lesions of myxedema, angiolymphoid hyperplasia, and generalized discoid lupus erythematosus and confirmed improvement in fibrotic lesions in these patients. Thus, we conclude that use of imiquimod improves fibrotic skin lesions. This report describes our experience with the treatment of this condition along with a review of the related literature.
Subject(s)
Humans , Acitretin , Angiolymphoid Hyperplasia with Eosinophilia , Cytokines , Fibrosis , Hyperplasia , Interferon-alpha , Lupus Erythematosus, Discoid , Myxedema , Skin , Tacrolimus , Toll-Like ReceptorsABSTRACT
Skin disease can be caused by high temperature, and it is related to the temperature regulation mechanism of human body, adaptation reaction to temperature change, and health problems due to the recent problematic climate change. In hyperthermia, hot and dry skin is typical manifestation, and sometimes the skin color turns red. On the other hand, the skin color can become pale in severe febrile convulsion. Burn is a skin damage caused by heat, and not only the skin but also the underlying tissues can be destroyed in severe case. It is important to determine the degree and extent of the burn to treat adequately. In the case of severe burns, systemic treatment and prevention of infection or shock should be needed. Miliaria, also called “sweat rash,†occurs when the sweat is accumulated as the sweat gland is closed and sweat cannot be secreted to the surface of the skin. The basis of treating miliaria is to keep the patient in a cool environment. Erythema ab igne is defined as a network of hyperpigmentation that occurs after prolonged exposure to heat that is not enough to cause burn. It may disappear when exposure to heat is interrupted, but it may remain permanently. The extent and mechanism of heat-induced skin disease very diverse and it should be carefully assessed for the severity of each disease, the treatment method and prognosis.
ABSTRACT
Skin diseases associated with athletic activities can be classified as skin infections, inflammatory reactions, trauma, and abnormal proliferation, depending on the cause of the condition. Athlete's nodule is a generic term for reactive nodules that occur in athletes. It is particularly common in the foot due to tight sneakers, repetitive pressure or friction, and inappropriate choice of shoes or other sports equipment. The diagnosis of black heel (calcaneal petechiae) should be considered when numerous black spots occur on the soles in patients who frequently engage in abrupt movements, such as starts, stops, or leaps. Palmoplantar eccrine hidradenitis may occur in athletes who play baseball, dance, and climb, activities in which repetitive and strong stimuli are applied to the floor of the hands and feet. Painful fat herniation should be suspected in cases of painful skin-colored firm nodules on the feet of athletes who place a large amount of weight on their feet when moving. Itching, urticaria, angioedema, chest tightness, and syncope occurring within 5 minutes after starting exercise should be suspected to be exercise-induced angioedema/anaphylaxis. Excessive force can cause deformation of nails, as in tennis toe and jogger's toenail. For the diagnosis and treatment of sports-related skin diseases, it is essential to pay attention to patients' hobbies and exercise habits, including sports, and to consider the relationship of those habits with the presumed mechanisms of the skin disease. In addition, thorough pre-exercise warm-ups, increasing strength gradually, and wearing proper equipment will help prevent the occurrence of sports-related skin diseases.