Neoplasms containing glomus cells are uncommon. Glomus cells within an angiomatosis, so called glomangiomatosis, is exceedingly rare with only three previously reported cases. We are describing the fourth case from a 17-year-old boy which involved his left hand, fingers, and distal forearm. We will review the previously reported cases.

We describe three patients with severe refractory cutaneous polyarteritis nodosa, resulting in painful ulcers involving the lower limbs and causing toe necrosis. Due to the severity of the cutaneous manifestations, the three patients received intravenous immunoglobulins at a dose of 1 g/kg/day for 2 days monthly. After the second intravenous immunoglobulin infusion, skin signs dramatically improved and completely healed after the third intravenous immunoglobulin infusion. Our findings indicate that intravenous immunoglobulins can be included in a therapeutic strategy to treat refractory cutaneous polyarteritis nodosa.

Introduction. - Anticoagulation clinics and computerized management of chronic oral anticoagulation increase the time spent in the therapeutic range with both mortality and morbidity reduction. Usually, anticoagulation clinics are hospital-based medical care centers. We report the five-year results from a general medicine center (CSCTA) using a computer-assisted management.Methods. - A prospective cohort observational study of 530 primary care patients that were receiving long term oral anticoagulation.Results. - Cardiac arrhythmia (55%), heart valve disease and venous thrombo-embolic disease (30%) represented the most common indications of oral anticoagulation. Patients received fluindione, warfarine and acenocoumarol in 80%, 13% and 7%, respectively. The duration of treatment was at least one year in 54% of the cases, and was at least three years in 25% of the cases. The rate of patients that were in average within the therapeutic range (INR 2-3) was 72%, while 12% were under and 16% over the therapeutic range. Corresponding rates were 82, 17 and 1% respectively for all anticoagulation targets (INR 1.5-4.5). Twenty-six bleeding events (4.9 per 100 patient-years) and four thrombotic complications (0.75 per 100 patient-years) occurred. Life-threatening hemorrhage occurred in 1.3 per 100 patient-years. After the equilibration of the anticoagulation. the average delay of control between two consecutive INR was 19 days.Conclusion. - The results obtained with CSCTA were similar to those reported by other anticoagulation clinics regarding hemorrhagic complications and time spent in the therapeutic range. In contrast, thrombotic events were less frequent. Because of the absence of a control group, a medico-economic analysis could not be performed. (C) 2009 Societe nationale francaise de medecine interne (SNFMI). Published by Elsevier Masson SAS. All rights reserved.

La mise en place de cliniques d’anticoagulants et d’aide à la gestion informatisée des traitements anti-vitamine K (AVK) a permis une amélioration du contrôle des International Normalized Ratios (INR) et son corollaire, une diminution de la mortalité et de la morbidité hémorragiques secondaires à la prise d’AVK. Nous rapportons les résultats obtenus depuis cinq ans au sein d’un centre de suivi et de conseil des traitements anticoagulants (CSCTA) développé en médecine libérale ambulatoire qui utilise un logiciel informatique d’aide à la prescription.Étude de suivi de cohorte regroupant 530 patients de médecine de ville (238 femmes) d’âge moyen 72 ans.Les troubles du rythme cardiaque (55 %), les valvulopathies et la pathologie thromboembolique (30 %) représentaient les principales indications d’anticoagulation. Les patients recevaient du fluindione (80 %), de la warfarine (13 %) ou de l’acénocoumarol (7 %). Les durées de traitement étaient dans 54 % des cas supérieures à un an et dans 25 % des cas supérieures à trois ans. Les patients passaient en moyenne 72 % du temps dans la cible thérapeutique (INR 2–3), 16 % au-dessus et 12 % au-dessous de cette cible. Ces pourcentages étaient respectivement de 82 %, 17 % et 1 % pour toutes cibles confondues (INR 1,5–4,5). Vingt-six hémorragies ont été répertoriées (4,9 épisodes hémorragiques pour 100 patients-année) dont 1,3 épisodes hémorragiques sévères pour 100 patients-année. Quatre accidents thrombotiques ont été rapportés (0,75 événements pour 100 patients-année). Le délai moyen de contrôle entre deux INR était de 19 jours pour les patients équilibrés.Les résultats du CSCTA sont conformes à ceux publiés par les cliniques d’anticoagulants en matière de temps passé dans la zone thérapeutique et de complications hémorragiques. L’absence de groupe témoin n’autorisait pas une analyse médico-économique qui aurait permis d’estimer le bénéfice réel de cette structure.Anticoagulation clinics and computerized management of chronic oral anticoagulation increase the time spent in the therapeutic range with both mortality and morbidity reduction. Usually, anticoagulation clinics are hospital-based medical care centers. We report the five-year results from a general medicine center (CSCTA) using a computer-assisted management.A prospective cohort observational study of 530 primary care patients that were receiving long term oral anticoagulation.Cardiac arrhythmia (55%), heart valve disease and venous thrombo-embolic disease (30%) represented the most common indications of oral anticoagulation. Patients received fluindione, warfarine and acenocoumarol in 80%, 13% and 7%, respectively. The duration of treatment was at least one year in 54% of the cases, and was at least three years in 25% of the cases. The rate of patients that were in average within the therapeutic range (INR 2–3) was 72%, while 12% were under and 16% over the therapeutic range. Corresponding rates were 82, 17 and 1% respectively for all anticoagulation targets (INR 1.5–4.5). Twenty-six bleeding events (4.9 per 100 patient-years) and four thrombotic complications (0.75 per 100 patient-years) occurred. Life-threatening hemorrhage occurred in 1.3 per 100 patient-years. After the equilibration of the anticoagulation, the average delay of control between two consecutive INR was 19 days.The results obtained with CSCTA were similar to those reported by other anticoagulation clinics regarding hemorrhagic complications and time spent in the therapeutic range. In contrast, thrombotic events were less frequent. Because of the absence of a control group, a medico-economic analysis could not be performed.

Background. We report a case of necrolytic migratory erythema in a patient with Waldmann's disease.Patients and methods. A 55-year-old male patient with a history of Waldmann's disease was hospitalized for a rash on the trunk and limbs comprising annular polycyclic lesions with peripheral scaling evocative of necrolytic migratory erythema. High-protein and fatty-acid-supplemented parenteral feeding led to rapid improvement of the patient's cutaneous lesions.Discussion. Waldmann's disease is characterized by intestinal lymphatic abnormalities leading to exudative intestinal disease causing protein loss in the bowel lumen and deficient fatty acid absorption. The pathogenesis of necrolytic migratory erythema is not fully understood. increased serum glucagon does not appear to be the only mechanism involved. The occurrence of necrolytic migratory erythema in a patient with Waldmann's disease supports the current physiopathological hypothesis of the role of decreased plasma protein and amino acid levels in necrolytic migratory erythema.

Resume Introduction Nous rapportons un cas d’erytheme necrolytique migrateur associe a une maladie de Waldmann. Observation Un homme de 55 ans,ayant une maladie de Waldmann depuis 20 ans, a ete hospitalise pour une eruption du tronc et des membres, faite de lesions annulaires a contours polycycliques avec collerette de desquamation peripherique, evocatrice d’un erytheme necrolytique migrateur. Une alimentation enterale hyperprotidique et enrichie en acides gras a entraine une amelioration rapide de son etat cutane. Discussion La maladie de Waldmann est caracterisee par des lymphangiectasies intestinales responsables d’une enteropathie exsudative a l’origine de pertes protidiques et d’un defaut d’absorption des acides gras.La physiopathologie de l’erytheme necrolytique migrateur est imparfaitement comprise. L’hyperglucagonemie ne semble pas etre le seul mecanisme en cause. La survenue d’un erytheme necrolytique migrateur au cours d’une maladie de Waldmann renforce les hypotheses physiopathologiques actuelles suggerant le role de l’hypoprotidemie et du deficit plasmatique en acides amines dans l’erytheme necrolytique migrateur.

Toxoplasmosis is a potentially serious fetal infection associated with maternal seroconversion of toxoplasmosis during pregnancy. Follow-up and treatment vary between different countries. We present a case of congenital toxoplasmosis with unusual physiopathology and symptomatology. The mother was immunized before the beginning of pregnancy but immunosuppressive treatments for Crohn disease maintained during the pregnancy could explain toxoplasmosis reactivation in the mother and congenital toxoplasmosis. The baby presented reversible B lymphopenia and hypogammaglobulinemia.

HistopathologyVolume 36, Issue 6 p. 566-567 Primary cutaneous carcinoid tumour P Courville, P Courville Departments of Pathology, Search for more papers by this authorP Joly, P Joly Dermatology andSearch for more papers by this authorE Thomine, E Thomine Departments of Pathology, Search for more papers by this authorJ Ziade, J Ziade Independent Pathology Laboratory,Rouen, France Search for more papers by this authorJ C Soubrane, J C Soubrane Departments of Pathology, Search for more papers by this authorJ M Kuhn, J M Kuhn Endocrinology,Rouen University Hospital, Charles Nicholle, and Search for more papers by this authorP Lauret, P Lauret Dermatology andSearch for more papers by this author P Courville, P Courville Departments of Pathology, Search for more papers by this authorP Joly, P Joly Dermatology andSearch for more papers by this authorE Thomine, E Thomine Departments of Pathology, Search for more papers by this authorJ Ziade, J Ziade Independent Pathology Laboratory,Rouen, France Search for more papers by this authorJ C Soubrane, J C Soubrane Departments of Pathology, Search for more papers by this authorJ M Kuhn, J M Kuhn Endocrinology,Rouen University Hospital, Charles Nicholle, and Search for more papers by this authorP Lauret, P Lauret Dermatology andSearch for more papers by this author First published: 25 December 2001 https://doi.org/10.1046/j.1365-2559.2000.00918-2.xCitations: 20Read the full textAbout ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL No abstract is available for this article.Citing Literature Volume36, Issue6June 2000Pages 566-567 RelatedInformation

INTRODUCTION:Monoclonal light and heavy chain deposition disease is a rare syndrome distinct from light chain amyloid, which is defined by the presence of monoclonal deposits of immunoglobulins in various tissues.CASE-REPORT:A 65-year-old man presented with renal symptoms due to membranoproliferative glomerulonephritis, associated with urticarial papules located on the arms and back. Histological examination of a skin biopsy specimen showed lymphocytic vasculitis. Direct immunofluorescence examination of kidney and skin lesions using anti-gamma 2 and anti-Kappa monoclonal antibodies, showed a similar staining on the basement membrane zone and vessel walls.COMMENTS:As far as we know, this is the first documentation of monoclonal light and heavy chain deposition disease associated with a lymphocytic skin vasculitis and renal involvement caused by similar monoclonal deposits of immunoglobulins in the kidney and skin.

Esophageal involvement during pemphigus vulgaris (PV) has rarely been reported. Moreover, this particular form of PV has only been studied by immunofluorescence. To determine the precise in vivo and in vitro ultrastructural location on esophageal cell membranes, of anti-intercellular substance (ICS) antibodies, we analyzed using direct and indirect immunoelectron microscopy (IEM), esophaqeal biopsy and serum samples from four patients with PV and esophageal involvement. Direct IEM on patients' esophageal mucosae showed that IgG deposits were strictly localized to separated desmosomal structures on the free surface of acantholytic esophageal cells. Indirect IEM of normal human esophageal mucosa labeled with sera from 3 patients demonstrated IgG deposits in the extracellar part of desmosomal regions but also along the esophageal cell plasma membrane. This study enabled the precise localization of esophageal anti-epithelial cell surface antibody deposits in patients with PV and esophageal involvement. Moreover, it suggests that the acantholytic process may begin in the interdesmosomal regions of the esophageal cell membrane.

A case of dermatitis herpetiformis in a 60-year-old woman with polycystic kidney disease on hemodialysis is reported. Bullous dermatoses associated with hemodialysis are analyzed.