Angiocentric T-cell lymphomas have been described as a distinctive clinicopathologic entity in the spectrum of peripheral T-cell lymphomas, with a prominent invasion of blood vessels by lymphomatous cells. In these conditions, the presence of Epstein-Barr virus (EBV) genomes has been demonstrated, suggesting that EBV might play a major role in their cause. Herein, we report a case of cutaneous angiocentric T cell lymphoma associated with the EBV. The patient was diagnosed with nasal angiocentric T cell lymphoma 5 months ago, and treated with cyclophosphamide, doxorubicin, vincristine, and prednisolone (CHOP) combination chemotherapy. After three cycles of CHOP, skin lesions developed. A skin biopsy specimen showed an angiocentric and angioinvasive infiltrate containing some atypical lymphocytes. EBV encoded RNA (EBER) was demonstrated in lesional skin by in situ hybridization.
Biopsy ; Blood Vessels ; Cyclophosphamide ; Doxorubicin ; Drug Therapy, Combination ; Genome ; Herpesvirus 4, Human* ; Humans ; In Situ Hybridization ; Lymphocytes ; Lymphoma, T-Cell* ; Lymphoma, T-Cell, Peripheral ; Prednisolone ; RNA ; Skin ; Vincristine

Afloqualone is a centrally acting muscle relaxant and has been used in therapy for musculotensive diseases. A 35-year-old female had been taking afloqulaone for a left hemifacial spasm for one month. The patient visited our hospital with erythematous papules and patches on the sun exposed area. A photopatch test with afloqualone ointment with the concentration of 0.1%, 1%, 5% turned out negative. An oral provocation test was also done and it showed a positive result with a decrease of MED to UVA (10 J/cm²).
Adult ; Dermatitis, Phototoxic* ; Female ; Hemifacial Spasm ; Humans ; Solar System

No Abstract Available.

The Carneys complex is a multisystemic tumorous disorder that features myxoma(heart, skin, and breast), spotty skin pigmentation(cutaneous and mucocutaneous), endocrine tumors(adrenal, testicular, and pituitary) and peripheral nerve tumors. The criteria far diagnosis of the complex is the presence of two or more of the above conditions. The condition is transmitted as an autosomal dominant trait. The most serious components of the syndrome are cardiac myxoma and psammomatous melanotic schwannoma. The cutaneous manifestations are a major clue in the disorder. We report a case of Carneys complex in a 19-year-old woman. She had spotty pigmentation on her face, left atrial myxoma, and myxoma on her right nipple. Her mother and sister had left atrial myxoma.
Diagnosis ; Female ; Humans ; Mothers ; Myxoma* ; Neurilemmoma ; Nipples ; Peripheral Nervous System Neoplasms ; Pigmentation ; Siblings ; Skin ; Young Adult

BACKGROUND: Cubitus varus and cubitus valgus deformities are common complications of distal humeral fractures in children. We evaluated the usefulness of supracondylar dome osteotomy as a treatment option for adults with cubitus varus or valgus deformity developed during childhood. METHODS: Ten patients who had received supracondylar dome osteotomy and stabilization with plates to treat cubitus varus or valgus deformity between July 2006 and August 2013 were included in this study. Their mean age at the time of surgery was 36.50 ± 10.22 years. The mean follow-up duration was 54.80 ± 32.50 months. We evaluated humerus-elbow-wrist angles (HEWA), improvements in the lateral prominence index (LPI) or medial prominence index (MPI), Mayo elbow performance scores (MEPS), and overall results in accordance with the Banerjee criteria. RESULTS: For the six patients with cubitus varus, the mean postoperative HEWA, mean correction angle, and mean improvement in LPI were 9.72°± 3.95°, 27.67°± 10.75°, and 6.92% ± 3.40%, respectively. For the four patients with cubitus valgus, the mean postoperative HEWA, mean correction angle, and mean improvement in MPI were 14.73°± 2.97°, 11.55°± 3.26°, and 11.33% ± 6.39%, respectively. There was no significant difference between postoperative and preoperative mean MEPS. The subjective ulnar nerve symptoms were alleviated in all patients. The overall results were excellent in six and good in four patients. CONCLUSIONS: This study suggests that supracondylar dome osteotomy with secure fixation using double plates may be useful in correcting cubitus varus or cubitus valgus deformity, yielding good functional outcomes in adults.
Adult* ; Child ; Congenital Abnormalities* ; Elbow ; Follow-Up Studies ; Humans ; Humeral Fractures ; Osteotomy* ; Ulnar Nerve

Improvement of lichen planus was achieved by 11 weeks of daily oral treatment with griseofulvin. The patient was 59-year-old male, has had hypertension and diabetes mellitus for 9 months, and history of various drug intake to these diseses for 6 months. Four months before first visit, symptome of lichen planus had developed suddenly. Because of treatment failure of oral antihistamine and topical steroid for 6 weeks, we began to use griseofulvin. Praspective studies are needed to better assess the affectiveness of griseafulvin in the treatment of lichen planus.
Diabetes Mellitus ; Griseofulvin* ; Humans ; Hypertension ; Lichen Planus* ; Lichens* ; Male ; Middle Aged ; Treatment Failure

Fibrohistiocytic tumors are a diverse group of benign and malignant soft tissue lesions, including dermatofibroma, dermatofibrosarcomaprotuberans, and malignant fibrous histiocytoma. On the clinical point of view, the distinction between benign and malignant lesions and malignancy grading is far more important. Therefore, we investigated 23 fibrohistiocytic tumors, using PCNA (PC10) which was a useful marker of proliferating activity, to differentiate the benign lesions from the malignant and correlate with other prognostic factors including tumor necrosis. cellularity, histologic grade, and mitotic counts. The results obtained were as follows 1) Positive tumor cells were clearly identified by the characteristic diffuse or granular nuclear staining. 2) The number of PCNA-positive tumor cells were 2.16+/-2.39% in dermatofibroma, 16.12+/-7.38% in dermatofibrosacoma protuberans, and 28.02+/-17.47% in the malignant fibrous histiocytoma. The numbers of PCNA-positive tumor cells in the malignant lesions higher than in the benign (p<0.001). 3) Deep seated, large size (>5 cm) and recurred or metastatic cases of MFH were more the high PCNA index (more than 20%) than the low index (less than 20%) groups. 4) PCNA index in MFHs had positive correlation with the number of mitotic counts (r=0.7582, p<0.001), cellularity (r=0.5908, p<0.05) and histologic grade (r=0.4164, p<0.05). These results suggested that reactivity on PCNA might assist in the distinction between benign and malignant lesions in fibrohistiocytic tumors, and could be a useful prognostic factor in the patients with malignant fibrous histiocytoma.
Neoplasm Metastasis

BACKGROUND: The central physiological derangement of hemorrhagic fever with renal syndrome (HFRS) caused by hantaan virus (HTNV) is a vascular dysfunction, manifested by hemorrhage, impaired vascular tone and increased vascular permeability. Platelet activating factor (PAF), whose actions are mediated through a specific receptor, is a potent bioactive lipid. PAF has diverse biological functions in the vascular system, such as increasing vascular permeability, adhesion of leukocytes to the endothelium and reduction of cardiac output, which result in hypotension and shock. The goal of the present study was to investigate whether PAF is involved in the pathogenesis of HFRS. For this purpose, we evaluated the effect of HTNV on the expression of PAF receptor (PAF-R) and on the activity of PAF-acetylhydrolase (PAF-AH) instead of PAF because PAF is rapidly degraded by PAF-AH in vivo. MATERIALS AND METHODS: To evaluate the expression of PAF-R, we performed reverse-transcription PCR, western blot and FACS analyses using HTNV-infected human umbilical vein endothelial cells (HUVECs) and non-infected (control) HUVECs. In addition, we measured the activity of plasma PAF-AH in HFRS patients and normal healthy persons. RESULTS: The mRNA and protein expression of PAF-R was increased in HTNV-infected HUVECs compared with control HUVECs at 2 and 3 days post-infection (d.p.i.). FACS analysis showed that HTNV induced the surface expression of PAF-R in HUVECs from 2 d.p.i. The activity of plasma PAF-AH was 2.5-fold lower in HFRS patients than in normal healthy persons. CONCLUSION: Increased PAF-R expression by HTNV might increase the responsiveness to PAF in endothelial cells. Reduced PAF-AH activity in the blood of HFRS patients might delay PAF degradation. These results suggest that changes in PAF-R and PAF-AH by HTNV might influence to PAF activity and might be involved in the vascular dysfunction of HFRS.
Blood Platelets ; Blotting, Western ; Capillary Permeability ; Cardiac Output ; Endothelial Cells ; Endothelium ; Hantaan virus ; Hemorrhage ; Hemorrhagic Fever with Renal Syndrome ; Human Umbilical Vein Endothelial Cells ; Humans ; Hypotension ; Leukocytes ; Plasma ; Platelet Activating Factor ; Platelet Membrane Glycoproteins ; Polymerase Chain Reaction ; Receptors, G-Protein-Coupled ; RNA, Messenger ; Shock

We report on a 28 years old female with recurred cardiac myxomas who presented with dizziness, headache, and blurred vision. She had an excision of biatrial atrial myxomas 10 years age. Varying sized multiple cerebral aneuysms and myxomas in left atrium and left ventricle were found on a cerebral angiogram and echocardiogram, respectively. After wide excision including interatrial septum and part of left ventricular septum, her symptoms were much improved.
Adult ; Dizziness ; Female ; Headache ; Heart Atria ; Heart Ventricles ; Humans ; Intracranial Aneurysm ; Myxoma* ; Ventricular Septum

BACKGROUND: Beta-adrenergic receptor Kinase 1(betaARK1) is a serine/threonine kinase attached, which inhibits the coupling of beta-adrenergic receptor with G-protein. Myocardial betaARK1 level is usually elevated in heart failure and hypertrophy, but it is not known whether the circulating betaARK1 level is related with the degree of cardiac hypertrophy. This study was performed to evaluate the association of the betaARK1 level in circulating mononuclear leukocytes(MNL) in untreated hypertension with left ventricular mass in hypertensive patients. Method: Nineteen non-treated hypertensive patients were included for this study. High blood pressure was confirmed when systolic BP is over 150 mmHg or diastoli BP is over 95 mmHg. Echocardiography was performed to evaluate the degree of hypertrophy by measuring the left ventricular mass index(LVMI) and relative wall thickness(RWT), and test the LV function by measuring the ejection fraction(EF) according to ASE guideline. At the same time, blood was collected from each patient and MNL were isolated by gradient centrifuge with Ficoll-400. Total RNA was purified from MNL and semi-quantitative RT-PCR was performed. After reverse transcription, PCR was done with primers for human betaARK1 and GAPDH as external control. betaARK1 levels were expressed by ratio to GAPDH level and estimated the relations with clinical and Echocardiographic parameters. Result: We studied confirmed 19 hypertensive patients(10 men and 9 women, mean age of 50.6 years). Echocardiographically measured indices(mean+/-SD) were as follows; LVMI(137.3+/-30.6g/m2), PWT(0.53+/-0.09) and EF(54.6+/-8.5%). Ratio of betaARK1 levels to GAPDH was from 0.10 to 0.96 (0.62+/-0.25). betaARK1 levels were correlated with LVMI(correlation coefficient: r=.502, p=.029) and RWT(r=.627, p=.004). But Systolic BP(r=0.009, p=.93), diastolic BP(r=.07, p=.85) or EF(r=.045, p=.84) were not related to level of betaARK1. CONCLUSIONS: The betaARK1 level of circulating MNL was correlated well with the degree of the cardiac hypertrophy estimated by LVMI and RWT. This data suggests that activation of sympatho-adrenal system would exert a major role in developing cardiac hypertrophy and we can expect the decreased responsiveness to catecholamine in the heart of hypertensive patients. betaARK1 in circulating MNL might be used as a predictor or marker for LV hypertrophy in hypertensive patients.
beta-Adrenergic Receptor Kinases ; Cardiomegaly ; Echocardiography ; Female ; GTP-Binding Proteins ; Heart ; Heart Failure ; Humans ; Hypertension ; Hypertrophy ; Leukocytes, Mononuclear* ; Male ; Phosphotransferases ; Polymerase Chain Reaction ; Reverse Transcription ; RNA