Intravenous immunoglobulins as treatment of severe cutaneous polyarteritis nodosa.

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.

[Anticoagulation clinics for outpatients: a 5-year experience]. / Expérience d'un centre de suivi et de conseil des traitements anticoagulants oraux en médecine de ville: résultats à cinq ans.

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, warfarin 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.

[Necrolytic migratory erythema in Waldmann's disease]. / Erythème nécrolytique migrateur au cours d'une maladie de Waldmann.

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.

[Light and heavy chain deposition disease with cutaneous and renal manifestations]. / Maladie des dépôts de chaînes lourdes et légères avec manifestations cutanées et rénales.

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.

Dermatitis herpetiformis and hemodialysis.

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.