Retroperitoneal castlemans disease associated with paraneoplastic pemphigus

Paraneoplastic pemphigus is a rare dermatologic condition that can occur as a paraneoplastic manifestation of lymphoproliferative disorders, visceral malignancies, Castleman disease and thymomas. In a pediatric age group paraneoplastic pemphigus has a striking association with Castleman disease and has a particularly poor prognosis in this age group. We describe a case of paraneoplastic pemphigus associated with retroperitoneal hyaline vascular variant of Castleman disease with a fatal outcome despite aggressive management

Clinico-radiological profile of arrhythmogenic right ventricular dysplasia at a tertiary care center: two year experience

Arrythmogenic right ventricular dysplasia [ARVD/C] refers to fibro fatty infiltration replacement of ventricular myocardium especially that of right ventricle. The clinical presentation varies from asymptomatic state to ventricular tachycardia, heart failure and even sudden death. Diagnosis is established using modified ARVD/C taskforce criteria. Among all the various modalities of diagnosis, magnetic resonance imaging [MRI] gives most comprehensive evaluation of both morphological and functional abnormalities in this disease. MRI may not only obviate need for myocardial biopsy but also give insights into the nature of disease like presence of left ventricular myocardial involvement. We present our 2 years experience of ARVD/C patents who were admitted in our center and in whom diagnosis of ARVD/C was supported by excellent MR imaging. This study was conducted by Department of Radiology and Cardiology SKIMS, a tertiary care center for a period of 2 years. Patients with suspected ARVD/C based on clinical, electrophysiological and echocardiographic findings were subjected to MR imaging. Patients were excluded if they had history metallic implants, claustrophobia or were uncooperative. In this study stress was laid on diagnostic role of MRI in ARVD/C. The median age at presentation was 31 years [range 21-43 years]. 80% of patients were males. Most common clinical presentation was palpatations [40%]. Syncope was present in 27% and heart failure in 13%. EKG suggestive of ARVD was seen in 87%. Echocardiographic features suggestive of ARVD/C was seen in all 15 patients. Family history of premature sudden death less than 35 years old was present in one patient only. MRI evidence classical for ARVD/C was seen in 80%. Demographic features and mode of presentation of our patients is consistent with what has been rest of the world. We performed MRI in all patients to increase the specificity of our diagnosis. MR imaging allows a three-dimensional evaluation of the right ventricle and provides the most important anatomic, functional, and morphologic criteria for diagnosis of ARVD/C within one single study. MR imaging appears to be the optimal imaging technique for detection and follow-up of clinically suspected ARVD/C

Uncomplicated spontaneous rupture of the pancreatic pseudocyst into the GUT- C T documentation: a series of two cases

Spontaneous rupture of the pancreatic pseudocyst into the surrounding hollow viscera is rare and, may be associated with life-threatening bleeding. Such cases require emergency surgical intervention. Uncomplicated rupture of pseudocyst is an even rarer occurrence. We present herein two cases of uncomplicated spontaneous rupture of a pancreatic pseudocyst into the stomach with complete resolution