摘要
Background@#Laugier-Hunziker syndrome (LHS) is a rare, benign, acquired disorder that is usually characterized by hyperpigmentation of the oral cavity and lips, along with longitudinal melanonychia. Till date, its characteristics have not been investigated in a large number of patients in a single-center study. @*Objective@#This study aimed to investigate the clinical, dermoscopic, and histological characteristics of LHS. @*Methods@#We retrospectively reviewed the medical records, biopsy specimens, and clinical and dermoscopic photographs of 21 patients diagnosed with LHS at Pusan National University Hospital (Busan and Yangsan) over a period of 15 years (2008∼2022). @*Results@#Among the 21 patients, 20 patients (95.2%) were female and one patient (4.8%) was male, and the mean age was 56.0 years (range: 18∼75 years). The lower lip (95.2%) was the most frequently affected oral mucosa, followed by the upper lip (81.0%), buccal mucosa (66.7%), tongue (57.1%), gingiva (42.9%), and palate (14.3%). The nails were the most commonly affected extraoral site, and in our study, eight of 21 patients (38.1%) presented with periungual lesions. Based on the dermoscopic findings, a globular pattern was the most common. Histologically, the lesions showed epithelial acanthosis and increased basal keratinocyte melanin without an increase in the number of melanocytes. @*Conclusion@#This is the first study to investigate the clinical, dermoscopic, and histological characteristics of patients with LHS. The results of this study could be helpful in understanding LHS.
摘要
Background@#Periorificial dermatitis (POD) is an acneiform or rosacea-like eruption that occurs in children and adults. Although POD is not rare, studies investigating its clinical characteristics and severity, particularly during childhood, have not been well conducted. @*Objective@#This study aimed to investigate the clinical findings and severity of POD and the differences between childhood and adult POD. @*Methods@#We retrospectively reviewed the medical records and clinical photographs of 131 patients diagnosed with POD in the Pusan National University Hospital and Pusan National University Yangsan Hospital over a 17-year period (2003∼2019). @*Results@#Among 131 patients, 23 (17.6%) were children and 108 (82.4%) were adults. The mean age was 9.0 years (2.4∼17 years) and 43.9 years (19∼79 years), respectively. The male-to-female ratio was lower in adults with POD (1:2.6) than in children with POD (1:0.9). The involvement of the periocular area was more frequent in childhood POD (47.8%) than in adult POD (17.6%), although the involvement rates of the perinasal, perioral, and extrafacial areas were not different. When we checked the PeriOral Dermatitis Severity Index (PODSI), it was higher in adults (4.3±1.5) than in children (3.2±2.0). The clinical course according to age, sex, treatment, and severity did not differ between the groups. @*Conclusion@#Although the sample size of childhood POD was small, this study identified that the periocular area was more frequently involved and PODSI was lower in childhood POD than in adult POD.
摘要
Background@#Currently, there is no consensus on the treatment of psoriasis in Korean patients. @*Objective@#This study aimed to establish a consensus on the basic therapeutic principles for Korean patients with plaque psoriasis. @*Methods@#Using the modified Delphi method, a steering committee proposed 53 statements for the first Delphi round, which covered five subjects: (1) the goal of treatment and evaluation of disease severity, (2) topical therapy, (3) phototherapy, (4) conventional systemic therapy, and (5) biologic therapy. The panel of dermatologists scored the level of agreement for each statement on a ten-point scale with scores ranging from 1 (strongly disagree) to 10 (strongly agree). After discussing the results of the first round, the committee reformulated 41 statements. Finally, consensus was defined as more than 70% of the second round scores being ≥7. @*Results@#The panel participants strongly agreed that the ideal treatment goals for Korean patients with plaque psoriasis should include complete skin clearance and high dermatological quality of life. A strong consensus was also reached on the use of topical agents for psoriasis of any severity, the consideration of phototherapy before biologics therapy, the conventional systemic agents for moderate-to-severe psoriasis, and the recommendation of biologic for retractable psoriasis to conventional systemic therapy and phototherapy. @*Conclusion@#This modified Delphi panel established an expert consensus on the therapeutic approach for Korean patients with plaque psoriasis. This consensus may improve the treatment outcomes for psoriasis in Korea.
摘要
Psoriasis is a common immune-mediated, inflammatory skin disease. However, unilateral psoriasis is rare and few cases have been reported. A 59-year-old male with a polio-affected right leg presented with exfoliative skin on the left leg for three months. He had been treated under the diagnosis of contact dermatitis with secondary infection. After the exfoliative skin lesions improved, psoriatic papules were noted, which was ultimately diagnosed as psoriasis and successfully treated with topical steroid and vitamin D agents. We propose that the Koebner phenomenon has acted as the triggering and aggravating factor of unilateral psoriasis in this patient.
摘要
Background@#Dupilumab is a human monoclonal antibody against interleukin-4 receptor α. Several clinical trials have demonstrated the rapid and excellent therapeutic effects of dupilumab. Although a growing number of studies have reported data on the real-world efficacy and safety of dupilumab for the treatment of atopic dermatitis, data on real-world experiences in Korea are limited. @*Objective@#To evaluate the real-world efficacy and safety of dupilumab for the treatment of moderate-to-severe atopic dermatitis in Korean patients. @*Methods@#This was a retrospective, single-center study. A total of 179 patients treated with dupilumab for at least 16 weeks were enrolled in this study. Based on electronic medical records, the clinical characteristics, Eczema Area and Severity Index (EASI) score, and adverse events were investigated. @*Results@#The mean baseline EASI score (26.5±7.2) significantly decreased at weeks 16, 40, 52, and 112 (p＜0.05). All and 55.2% of patients achieved EASI75 and EASI90 responses at week 52, and all and 75.0% of patients achieved EASI75 and EASI90 responses at week 112, respectively. Common adverse events were facial erythema (31.8%), conjunctivitis (24.0%), and herpes simplex virus infection (11.2%). Three serious adverse events of severe conjunctivitis, mycosis fungoides, and mesenteric venous thrombosis resulted in discontinuation of dupilumab. @*Conclusion@#Dupilumab was effective in real-world clinical practice with a favorable safety profile in Korean patients with moderate-to-severe atopic dermatitis.
摘要
Background@#Risankizumab, human immunoglobulin G1λ monoclonal antibody, selectively blocks the p19 subunit of interleukin-23-mediated signaling pathway. Risankizumab has emerged as an effective and well-tolerated therapeutic option for moderate-to-severe psoriasis. However, real-world data on treatment outcomes of risankizumab in Korean patients with psoriasis are limited. @*Objective@#To investigate the efficacy and safety of risankizumab in Korean patients with moderate-to-severe plaque psoriasis. @*Methods@#We retrospectively reviewed patients with moderate-to-severe psoriasis who were treated with risankizumab in our hospital from July 2020 to December 2022. A total of 45 patients with psoriasis who were treated with risankizumab for at least 16 weeks were included in this study. Patient demographics, Psoriasis Area and Severity Index (PASI) scores, Investigator’s Global Assessment scores, and adverse events were assessed. @*Results@#The mean baseline PASI score (13.1±7.7) was significantly decreased after 16 weeks of risankizumab treatment (0.9±1.4) (p＜0.05). Of the 45 patients, 40 showed a PASI 90 response at the last follow-up visit. No serious adverse events were observed. Three patients (6.7%) experienced mild adverse events such as injection site reaction, easy bruising, and headache. @*Conclusion@#Our real-world data demonstrated that risankizumab treatment is effective and safe in Korean patients with moderate-to-severe plaque psoriasis.
摘要
Psoriasis is a common immune-mediated skin disease. Myasthenia gravis and Graves’ disease are autoimmune diseases caused by autoantibodies against receptors located at the neuromuscular junction and thyroid-stimulating hormone receptor, respectively. A 29-year-old male presented with recurrent multiple erythematous scaly papules and plaques on his legs that had persisted for 3 years. A diagnosis of plaque psoriasis was made based on clinicopathological findings. The patient had a 26-year history of ocular myasthenia gravis, for which he had not been received any specific treatment. He also had a long history of Graves’ disease. To the best of our knowledge, psoriasis with myasthenia gravis and Graves’ disease has not been reported previously in the literature. Herein, we describe our examination of the causes of concurrent psoriasis, myasthenia gravis, and Graves’ disease.
摘要
Diffuse large B-cell lymphoma (DLBCL) is the most common type of non-Hodgkin’s lymphoma accounting for approximately one-third of all cases. DLBCL can present as a lymph node or extranodal tumor. Cavernous sinus (CS) is a small but complex structure in which various arteries, sympathetic plexuses, and cranial nerves are passing through. Cavernous sinus syndrome (CSS) results from any disease process that affects CS including tumor, vascular disease, infection, or inflammation. Herein, we report a case of extranodal DLBCL diagnosed by skin biopsy presenting as CSS. A 58-year-old male presented with a 3-week-old, gradually growing subcutaneous nodule on the left upper lip. He also suffered from ptosis, ophthalmoplegia, diplopia, and headache confined to the right side for 3 months. Histopathologic examination of the left upper lip showed dense dermal infiltration of atypical large tumor cells resembling centroblasts and immunoblasts. Immunohistochemistry studies revealed that the tumor cells were positive for CD20, BCL2, BCL6, MUM1, and MYC. After additional radiologic evaluation with positron emission tomography-computed tomography (PET-CT), brain magnetic resonance imaging, and orbital CT, he was finally diagnosed with extranodal DLBCL involving the right CS, oculomotor muscles, and left upper lip.
摘要
Cutaneous Rosai-Dorfman disease (RDD) is a rare, benign condition mainly affecting the lymph nodes and sometimes involving the skin. RDD is notorious for its poor response to medication, and surgery is often the preferred treatment option for limited lesions. Herein, we present a case of cutaneous RDD in a 49-year-old woman with multiple facial nodules that showed minimal response to treatment with steroids, isotretinoin, and methotrexate.The diagnosis of cutaneous RDD was confirmed based on histopathological examination. The patient showed minimal response to medication; however, electrosurgery resulted in a relatively good cosmetic response. The present case demonstrates that electrosurgery can be an effective treatment option for cutaneous RDD, especially in case of patients with multiple lesions. The rarity of this disease and the limited reports on its management highlights the need for further research on the efficacy of different surgical modalities for cutaneous RDD.
摘要
Background@#Scar sarcoidosis (SS), a rare form of cutaneous sarcoidosis, develops from preexisting scars. Owing to its rarity, the clinicopathologic features and its significance in clinical prognosis have been obscure. @*Objective@#This study aimed to investigate clinical, laboratory and histopathologic findings and to clarify characteristics associated with the development of SS and systemic involvement. @*Methods@#We retrospectively assessed clinical, laboratory and histopathologic findings of SS. Clinical factors including demographics, anatomic area, number of lesion (single, multiple), presence of symptoms, latent period, injury types related to scar and the proportion of systemic involvement were investigated. @*Results@#Of the 21 patients with SS, skin lesions appeared predominantly in females (85.7%) and in the head and neck (57.1%). The mean latent period was 163.5 months and 13 patients (61.9%) had multiple lesions. Injury types were varied, with no specific type identified as associated with SS. Histologically, discrete sarcoidal granulomas surrounded by densely packed collagen bundles with a thickening of numerous fibers were observed. Ten patients (47.6%) had systemic involvement and showed significantly more of the multiple lesions, longer latent period and higher level of mean serum angiotensin-converting enzyme than those without systemic involvement. @*Conclusion@#Various causes of scar were related to SS, but no specific injury type was identified as leading to SS. Although the exact pathomechanism remains unclear, the possibility of systemic involvement could be considered when the patients have multiple lesions, longstanding scars, and elevated serum angiotensin-converting enzyme.
摘要
Large-cell transformation of mycosis fungoides (LCT-MF) is an advanced stage of primary cutaneous T-cell lymphoma with a poor prognosis. Therapeutic options for these patients are often limited, and so far, they are not promising. An 81-year-old woman with a previous history of mycosis fungoides presented with aggravation of generalized erythematous scaly patches and new onset of ulcerated tumor on the abdomen for 3 months.Histopathological examination revealed a dense dermal infiltrate composed of atypical large lymphocytes. Immunohistochemically, the tumor cells were positive for CD30 expression. A diagnosis of CD30＋ LCT-MF was established. She was intensively treated with methotrexate (1 month), acitretin (1 month), and rituximab with dose-modified cyclophosphamide, doxorubicin, and prednisone (1 cycle). Despite such treatments, the improvement was minimal. Subsequently, the patient was started on brentuximab vedotin, 1.8 mg/kg intravenously once every 3weeks. She responded well to brentuximab therapy, and the skin lesions completely subsided within 12 weeks of treatment.
摘要
Background@#Ustekinumab is a humanized monoclonal antibody targeting interleukin (IL)-12 and IL-23. Although popular, data on its long-term efficacy and safety in Korean patients with psoriasis are limited. @*Objective@#To evaluate the long-term efficacy and safety of ustekinumab in Korean patients with psoriasis. @*Methods@#A retrospective study in patients with moderate-to-severe psoriasis who had been treated with ustekinumab for at least 5 years was conducted. The sex, age, body mass index, medical records, previous psoriasis therapy, psoriasis area and severity index (PASI) scores, and adverse events were evaluated. @*Results@#Twenty-five patients (median age 47.00 years) had been treated with ustekinumab for 5 years. The mean duration of psoriasis was 19.12±8.45 years, and the treatment duration with ustekinumab was 285.12±8.48 weeks. The baseline PASI was 17.52±7.38. PASI75 was achieved in 84% of the patients at week 28, and 96% of the patients maintained PASI75 during the 5-year follow-up period. Furthermore, 56% of patients reached PASI90 at 28 weeks, and 48% of patients maintained PASI90 for 5 years. No unexpected adverse events other than herpes zoster, herpes simplex, or elevated liver enzymes were reported. @*Conclusion@#Ustekinumab demonstrated long-lasting efficacy with an acceptable safety profile in Korean patients with moderate-to-severe psoriasis.
摘要
Immunoglobulin G4 (IgG4)-related disease is a systemic disease characterized by dense lymphoplasmacytic infiltrates with abundant IgG4-positive plasma cells and fibroblast proliferation. The retroperitoneal involvement of IgG4-related disease usually appears as a soft-tissue mass covering the abdominal aorta or entrapping the ureters, resulting in hydronephrosis. Here, we present a case of IgG4-related disease with retroperitoneal involvement in a 75-yearold woman with an unusual manifestation. A preoperative computed tomography (CT) scan revealed an irregular infiltrative retroperitoneal mass invading the normal anatomic barriers, raising the suspicion of malignancy or inflammation. Contrast-enhanced CT revealed a homogeneous progressive enhancement of the mass.
摘要
Renal epithelioid angiomyolipoma (EAML) is a rare variant of angiomyolipoma (AML), with a prominent epithelioid component. EAML usually presents as a large heterogeneous soft tissue lesion with intratumoral hemorrhage and variable necrosis or cystic changes. We present a case of multiloculated cystic renal EAML mimicking renal cell carcinoma in a 64-year-old female.Intracystic massive hemorrhage, hyperattenuating wall and septa on an unenhanced study, and enlarged intratumoral vessels can be helpful imaging features for distinguishing renal EAML from renal cell carcinoma.
摘要
Background@#Cyclosporine is a recommended third-line treatment for chronic spontaneous urticaria (CSU) that is resistant to H1-antihistamines according to the EAACI/GA 2 LEN/EDF/WAO guidelines for management of urticaria. However, some patients with refractory urticaria do not respond to cyclosporine or antihistamines. Omalizumab, a humanized anti-immunoglobulin E antibody, has been shown to be effective and safe for antihistamine-resistant CSU. However, there are few reports on the efficacy of omalizumab in patients with CSU who are resistant to cyclosporine. @*Objective@#To evaluate the efficacy of omalizumab in patients with cyclosporine-resistant CSU. @*Methods@#Recalcitrant CSU patients who had symptoms (seven-day urticaria activity score, UAS7≥7) despite being administered cyclosporine (3∼5 mg/kg/day) and H1-antihistamine at up to a four-fold increased dose for 4 weeks were included in this study. Omalizumab was administered at 150 mg or 300 mg by subcutaneous injection every 4 weeks. Efficacy was assessed using UAS7 12 weeks after the initial administration of omalizumab. @*Results@#A total of 28 patients (18 women, 10 men) with an average age of 43.8 years were included in the study. The mean duration of CSU was 40.0 (2∼288) months, and the mean UAS7 at baseline was 14.2 (9∼35) months. Overall, 22 patients (78.6%) showed a complete (UAS7=0) or partial response (0＜UAS7≤6) at 12 weeks. Patients who were administered 300 mg of omalizumab had a more complete response (9/15, 60%) than those who were treated with 150 mg (3/13, 23.1%). @*Conclusion@#Omalizumab is an effective therapy for CSU patients who do not respond to cyclosporine.
摘要
Immunoglobulin G4 (IgG4)-related disease is a systemic disease characterized by dense lymphoplasmacytic infiltrates with abundant IgG4-positive plasma cells and fibroblast proliferation. The retroperitoneal involvement of IgG4-related disease usually appears as a soft-tissue mass covering the abdominal aorta or entrapping the ureters, resulting in hydronephrosis. Here, we present a case of IgG4-related disease with retroperitoneal involvement in a 75-yearold woman with an unusual manifestation. A preoperative computed tomography (CT) scan revealed an irregular infiltrative retroperitoneal mass invading the normal anatomic barriers, raising the suspicion of malignancy or inflammation. Contrast-enhanced CT revealed a homogeneous progressive enhancement of the mass.
摘要
Renal epithelioid angiomyolipoma (EAML) is a rare variant of angiomyolipoma (AML), with a prominent epithelioid component. EAML usually presents as a large heterogeneous soft tissue lesion with intratumoral hemorrhage and variable necrosis or cystic changes. We present a case of multiloculated cystic renal EAML mimicking renal cell carcinoma in a 64-year-old female.Intracystic massive hemorrhage, hyperattenuating wall and septa on an unenhanced study, and enlarged intratumoral vessels can be helpful imaging features for distinguishing renal EAML from renal cell carcinoma.
摘要
Background@#Cyclosporine is a recommended third-line treatment for chronic spontaneous urticaria (CSU) that is resistant to H1-antihistamines according to the EAACI/GA 2 LEN/EDF/WAO guidelines for management of urticaria. However, some patients with refractory urticaria do not respond to cyclosporine or antihistamines. Omalizumab, a humanized anti-immunoglobulin E antibody, has been shown to be effective and safe for antihistamine-resistant CSU. However, there are few reports on the efficacy of omalizumab in patients with CSU who are resistant to cyclosporine. @*Objective@#To evaluate the efficacy of omalizumab in patients with cyclosporine-resistant CSU. @*Methods@#Recalcitrant CSU patients who had symptoms (seven-day urticaria activity score, UAS7≥7) despite being administered cyclosporine (3∼5 mg/kg/day) and H1-antihistamine at up to a four-fold increased dose for 4 weeks were included in this study. Omalizumab was administered at 150 mg or 300 mg by subcutaneous injection every 4 weeks. Efficacy was assessed using UAS7 12 weeks after the initial administration of omalizumab. @*Results@#A total of 28 patients (18 women, 10 men) with an average age of 43.8 years were included in the study. The mean duration of CSU was 40.0 (2∼288) months, and the mean UAS7 at baseline was 14.2 (9∼35) months. Overall, 22 patients (78.6%) showed a complete (UAS7=0) or partial response (0＜UAS7≤6) at 12 weeks. Patients who were administered 300 mg of omalizumab had a more complete response (9/15, 60%) than those who were treated with 150 mg (3/13, 23.1%). @*Conclusion@#Omalizumab is an effective therapy for CSU patients who do not respond to cyclosporine.
摘要
Tumor necrosis factor (TNF) antagonists are highly effective treatments for psoriasis. These agents provide the opportunity to improve disease activity and achieve clinical remission. Despite its efficacy, long-term use of biologics is associated with high financial costs and possibly life-threatening adverse events. Recently, there has been an increasing interest in discontinuing TNF antagonists in patients with psoriasis who have achieved a positive clinical response. However, there is a paucity of data and clinical guidelines concerning the cessation TNF antagonists in psoriasis treatment. Several factors, including cost, subsequent treatment efficacy, relative risks, and tolerability, should be considered before the decision is made to discontinue TNF antagonists. Well-designed clinical trials are necessary to identify factors that may trigger disease exacerbation after medication discontinuation in order to recognize the potential disadvantages of discontinuing treatment in patients who are previously successfully managed on TNF antagonists.
Subject(s)
Humans , Biological Products , Disease Progression , Psoriasis , Treatment Outcome , Tumor Necrosis Factor-alpha摘要
No abstract available.