Renal cell carcinomas (RCC) account for 2.4% of all adult malignancies. RCC is known for its aggressive nature, with approximately one-third of patients presenting with metastasis at the time of diagnosis. Cutaneous metastasis is a rare presentation of this cancer. A 78-year-old male presented with numerous erythematous nodules of various sizes on the left flank, which he had had for a month. He had undergone a left partial nephrectomy for papillary RCC 3 years previously and had been receiving chemotherapy since surgery. Histopathology revealed papillary architecture with prominent atypical nucleoli throughout the dermis. Immunohistochemical findings were positive for CD10, cytokeratin 7, PAX-8, vimentin, and alpha-methylacyl-CoA racemase. These results led to a diagnosis of cutaneous metastases of papillary renal cell carcinoma. Papillary RCC rarely presents with cutaneous metastases.Because these lesions are easily accessible, cutaneous lesions in patients with risk factors for RCC should be excised and pathologically evaluated.

Background: Since the disease burden of atopic dermatitis (AD) understandably increases with its disease severity, studies, especially on the baseline characteristics of severe AD, have been conducted in some countries to deepen the understanding of the disease and, by extension, formulate a relevant national policy. However, research on the baseline characteristics of severe AD in Korea remains insufficient. Objective: To report the baseline demographics and describe the clinical characteristics of adult Korean patients with severe AD, including medical and treatment history, clinical manifestations, disease severity, and laboratory findings, in order to understand the characteristics of severe AD. Methods: A single-center, prospective, non-interventional, observational, longitudinal study of 108 patients with severe AD was conducted between January 2021 and December 2022. Clinical data and patient-reported measures of signs and symptoms of AD were recorded at baseline. Results: The mean Eczema Area and Severity Index score of the patients was 28.5±6.8. The mean pruritus numerical rating scale, Patient-Oriented Eczema Measure, Dermatology Life Quality Index, and Atopic Dermatitis Control Tool scores were 8.1±1.7, 23.6±4.8, 21.9±6.6, and 19.5±3.9, respectively. Although a larger proportion of the headeck and trunk are affected than both limbs, the key signs of AD were less severe in the headeck region than in the other regions. Erythema and lichenification were more representative clinical signs of severe AD than induration and excoriation. The baseline data on previous treatments reflected the AD treatment guidelines in Korea. Conclusion This real-world data will provide dermatologists with a better understanding of severe AD, which will eventually lead to better clinical and health policy decisions for patients with severe AD.

Background: Atopic dermatitis (AD) patients usually wonder if their condition will worsen after vaccination or if they should continue with the treatment they are receiving. Considering that many patients treated with dupilumab had previously experienced severe AD symptoms and flares, the concerns are more understandable. Objective: This study aimed to investigate the safety of the coronavirus disease 2019 (COVID-19) vaccination in patients with AD treated with dupilumab. Methods: We enrolled 133 patients (101 dupilumab-treated and 32 systemic oral agentstreated as control group) with AD from six hospitals. Patients were asked about worsening pruritus and AD (5-point Likert scale) after vaccination. AD variables (eczema area and severity index [EASI], investigator’s global assessment [IGA], itch numerical rating scale [NRS], sleep NRS, and patient-oriented eczema measure [POEM]) were compared pre- and postvaccination. Adverse reactions to the COVID-19 vaccination were observed. Results: The incidence of adverse reactions to COVID-19 vaccines and worsening AD symptoms in dupilumab-treated patients were not significantly different compared with that in the control group. The itch NRS score increased significantly after vaccination (p<0.001).However, there were no statistically significant differences between the pre-and post-EASI, IGA, and POEM scores. Eight patients (7.9%) had worse EASI scores and required rescue therapy; however, most were easily managed with low-dose steroids or topical agents. None of the patients discontinued dupilumab treatment. Conclusion No serious adverse reactions were observed in patients with AD after COVID-19 vaccination. Exacerbation of pruritus and AD symptoms was observed but was mostly mild and transient.

Tuberculous lymphadenitis is among the most frequent presentations of extrapulmonary tuberculosis; the most common presentation is isolated chronic non-tender lymphadenopathy in young adults without systemic symptoms. Dupilumab is a fully human monoclonal antibody directed against interleukin-4 receptor-α that blocks the synergistic effects of interleukin-4 and interleukin-13 on allergic inflammation. Its well-known adverse events are allergic conjunctivitis, injection site reaction, and dupilumab facial redness. A 32-year-old female with severe atopic dermatitis was treated with dupilumab for 2 months at our clinic. She complained of multiple enlarged palpable lymph nodes on the right side of the neck and inguinal area for 2 months. Laboratory tests showed an increased total eosinophil count and immunoglobulin E level, as well as positive interferon-γ release assays. Radiological examination showed multiple low echoic and heterogeneous well-enhancing lymph nodes in level II, III, IV, and V of the neck. Histological examination revealed caseous necrosis and tuberculoid granuloma. The lymph node enlargements were completely relieved after antituberculosis treatment. The mechanism for the development of tuberculous lymphadenitis in a patient receiving dupilumab is not fully understood yet. In some previous studies, treatment with dupilumab suppressed the expression of genes related not only to T helper 2 and eosinophil response but also to proinflammatory responses. It could not inhibit the intracellular growth of Mycobacterium tuberculosis in macrophages, predisposing them to the development of tuberculous infection. To the best of our knowledge, this is the first report on the development of tuberculosis lymphadenitis in a patient treated with dupilumab.

Kaposi sarcoma (KS) is a vascular and lymphatic neoplasm caused by human herpesvirus 8 (HHV-8). AIDS-related KS has variable clinical courses ranging from mild disease presenting as an incidental finding to severe disease presenting as an aggressively progressing neoplasm that can lead to poor prognosis or even death. Typical clinical manifestation of KS is known as multiple cutaneous lesions on the extremities, trunk, and face with mucosal involvement. A 46-year-old male with AIDS complained of an erythematous patch on the right forearm which appeared 5 months ago. For a year, he was treated with antiretroviral drugs for AIDS. Physical examination revealed a 2.5-cm solitary erythematous patch only on the right forearm. Laboratory data revealed human immunodeficiency virus (HIV)-1 RNA of less than 40 copies/ml and a CD4 cell count of 264 cells/mm 3 . Histological examination revealed numerous slit-like spaces and vascular proliferation with primitive blood vessels dissecting between the collagen bundles and the dermis. Immunohistochemical staining showed positive HHV-8 nuclear staining of spindle cells. The histological features and positive HHV-8 immunohistochemical stain were consistent with the diagnosis of early patch stage of AIDS-related KS. KS can readily be misdiagnosed in early patch stage even by experienced clinicians, which leads to requirement of pathologic determination. On close inspection, it can be distinguished from other mimickers by its distinctive histologic features and immunohistochemical staining for HHV-8. Therefore, in cases of HIV-positive patients with clinically or histologically vascular-appearing mucocutaneous lesions, KS should be considered as a possible differential diagnosis.

Elastosis perforans serpiginosa (EPS) is a rare condition that belongs to the group of acquired perforating dermatosis. It usually appears as keratotic papules in serpiginous configuration with central atrophy on the head, nape, and extremities. It is characterized by the transepidermal elimination of elastic fibers with clumping and vertical orientation of elastic fibers. In many cases, it is associated with genetic or connective tissue diseases. A 26-year-old female patient with Down syndrome presented with brownish keratotic papules on both arms. The lesion had been present for 2 years, but there were no symptoms. The biopsy specimen showed transepidermal elimination of the degenerated elastic fibers. Verhoeff elastic staining revealed an increased number of thick elastic fibers. Based on these findings, the patient was diagnosed with EPS. Treatment with CO 2 laser was performed with a successful outcome.

In acute pancreatitis, colonic complications such as mechanical obstruction, ischemic necrosis, hemorrhage, and fistula are rare but their outcomes are fatal. It is known that colonic obstruction in acute pancreatits is more likely found in splenic flexure and transverse colon caused by severe inflammation of body and tail of pancreas leading to pressure necrosis. A 43-year-old man presented with abdominal distension lasting for 2 weeks. The patient had been admitted to our institution 6 weeks prior to the current admission, and the abdominal CT scan performed during the first admission revealed the pancreatic enlargement with peri-pancreatic fatty infiltration and fluid collection. At that time he was diagnosed as acute pancreatitis. The conservative management resulted in clinical improvent so that the patient was discharged. Upon the second admission, abdominal CT scan revealed multiple pseudocysts in the tail portion of pancreas with concominant wall thickening and narrowing of the proximal descending colon, and a dilatation of the bowel proximal to the splenic flexure. An obstruction of the descending colon as a complication of acute pancreatitis was suspected and the patient underwent left hemicolectomy. Abdominal distension was relieved after the operation and he was discharged on the 15th hospital days.
Acute Disease ; Adult ; Colectomy ; Colonic Diseases/*diagnosis/etiology/surgery ; Diagnosis, Differential ; Humans ; Intestinal Obstruction/*diagnosis/etiology/surgery ; Male ; Pancreatitis, Alcoholic/*complications/diagnosis ; Tomography, X-Ray Computed

PURPOSE: Acute lymphoblastic leukemia (ALL) accounts for approximately 75% of all cases of childhood leukemia. We investigated epidemiology, clinical and laboratory features and treatment outcome of the children with ALL in Korea during recent 5 years. METHODS: One thousand forty nine patients were enrolled between January 1994 and December 1998 from 37 major hospitals in Korea. The data regarding the clinical and laboratory features including age, WBC counts at diagnosis, immunophenotype, morphology, cytogenetics and treatment outcome of patients were analyzed retrospectively by review of patient's medical records. Kaplan-Meier survival curves were constructed. The differences between groups analyzed by log-rank test. RESULTS: There were 597 males and 452 females. The distribution between the age 2 and 5 years is most common in 46.1%. The annual incidence rate per 100,000 population varied from 1.6 to 2.2. The 5 year event free survival (EFS) rates according to good prognostic factors were as follows: 67% bet ween 1-9 year of age at diagnosis, 69% in under 10,000/mm3of initial WBC count, 74% in early pre-B cell CALLA ( ) immunophenotype, 65% in L3 morphology, 68% in no CNS invasion. Most of patients were treated by CCG treatment protocol. The 5 year EFS was 63%. Main complications were sepsis (21.8%) and hemorrhage (12.5%). The relapse rate was 15.6%. The common causes of death were sepsis, DIC, pneumonia, relapse. CONCLUSION: Our results could provide the most recent and important information about acute lymphoblastic leukemia of children in Korea.
Cause of Death ; Child ; Clinical Protocols ; Cytogenetics ; Dacarbazine ; Diagnosis ; Disease-Free Survival ; Epidemiology* ; Female ; Hemorrhage ; Humans ; Incidence ; Kaplan-Meier Estimate ; Korea* ; Leukemia ; Male ; Medical Records ; Pneumonia ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Precursor Cells, B-Lymphoid ; Recurrence ; Retrospective Studies ; Sepsis ; Treatment Outcome

In orthodontics and orthognathic surgery, cephalogram has been routine practice in diagnosis and treatment evaluation of craniofacial deformity. But its inherent distortion of actual length and angles during projecting three dimensional object to two dimensional plane might cause errors in quantitative analysis of shape and size. Therefore, it is desirable that three dimensional object is diagnosed and evaluated three dimensionally and three dimensional CT image is best for three dimensional analysis. Development of clinic necessitates evaluation of result of treatment and comparison before and after surgery. It is desirable that patient that was diagnosed and planned by three dimensional computed tomography before surgery is evaluated by three dimensional computed tomography after surgery, too. But Because there is no standardized normal values in three dimension now and three dimensional Computed Tomography needs expensive equipments and because of its expenses and amount of exposure to radiation, limitations still remain to be solved in its application to routine practice. If postoperative three dimensional image is constructed by pre and postoperative lateral and postero-anterior cephalograms and preoperative three dimensional computed tomogram, pre and postoperative image will be compared and evaluated three dimensionally without three dimensional computed tomography after surgery and that will contribute to standardize normal values in three dimension. This study introduced new method that computer-simulated three dimensional image was constructed by preoperative three dimensional computed tomogram and pre and postoperative lateral and postero-anterior cephalograms, and for validation of new method, in four cases of dry skull that position of mandible was displaced and four patients of orthognathic surgery, computer-simulated three dimensional image and actual postoperative three dimensional image were compared. The results were as follows. 1. In four cases of dry skull that position of mandible was displaced, range of displacement between computer-simulated three dimensional images and actual postoperative three dimensional images in co-ordinates values was from -1.8 mm to 1.8 mm and 94% in displacement of all co-ordinates values was from -1.0 mm to 1.0 mm and no significant difference between computer-simulated three dimensional images and actual postoperative three dimensional images was noticed(p>0.05). 2. In four cases of orthognathic surgery patients, range of displacement between computer-simulated three dimensional images and actual postoperative three dimensional images in co-ordinates values was from -6.7 mm to 7.7 mm and 90% in displacement of all co-ordinates values was from -4.0 to 4.0 mm and no significant difference between computer-simulated three dimensional images and actual postoperative three dimensional images was noticed(p>0.05). Conclusively, computer-simulated three dimensional image was constructed by preoperative three dimensional computed tomogram and pre and postoperative lateral and postero-anterior cephalograms. Therefore, potentiality that can construct postoperative three dimensional image without three dimensional computed tomography after surgery was presented.
Congenital Abnormalities ; Diagnosis ; Humans ; Imaging, Three-Dimensional ; Mandible ; Orthodontics ; Orthognathic Surgery* ; Reference Values ; Skull

BACKGROUND: Lamotrigne (LTG) is a newly developed antiepileptic drug which has shown to'be effective for medically intractable partial seizures. LTG was recently introduced to Korea but its clinical efficacy has not been investigated yet. METHODS: We assigned 34 medically intractable localization related epileptic patients taking maximally tolerable dose of carbarmazepine(CBZ). The study protocol consisted of 12 weeks of baseline phase, 4 weeks of phase I (drug -adjustment phase) and 8 weeks o f phase II (maintenance of LTG 200mg/day) After phase II, eligible patients entered into long-term therapy. Two patients dropped out during phase II for adverse event (AE) in one, and AE and poor seizure control in the other. RESULTS: Intention to treat analysis of the seizure outcome after 12 weeks of LTG add-on therapy showed mean seizure frequency reduction of 23.6% (p=O.006). More than 50% seizure frequency reduction was seen in 9 of 34 patients (26.4%), which was comparable to the results of previous clinical trials. On the other hand, the incidence of AE were quite high, which was developed in 27 patients. Dizziness with or without blurred vision and/or diplopia were the most common AE and occurred in 64.7%, which improved promptly by either reduction of CBZ or LTG doses. Twenty-two patients entered into long-term therapy and 18 patients showed either maintenance or more. LTG add-on therapy did not show any significant alterations of baseline Lab. Tests. DISCUSSION: LTG was an effective and safe new antiepileptic drug. However, about two-third of our patients developed A. E. similar to CBZtoxicity, which should be carefully considerd for treating patients taking maximally tolerable CBZ therapy. The proportion of patients taking LTG 300mg/day or more was very low in this study, which suggested the racial difference of tolerability to LTG.
Carbamazepine* ; Diplopia ; Dizziness ; Hand ; Humans ; Incidence ; Intention to Treat Analysis ; Korea ; Seizures