[Langerhans cell histiocytosis and Erdheim-Chester disease, a continuity?] / Histiocytose langerhansienne et maladie d'Erdheim-Chester, une continuité ?

INTRODUCTION: Erdheim-Chester disease and langerhans cell histiocytosis are two rare diseases separate on clinical, radiological and histological aspects. However, several cases involving both entities have been described. OBSERVATION: A 70-year-old man had a central diabetes insipidus, xanthelasmas, retroperitoneal fibrosis and osteosclerosis of the legs suggestive of Erdheim-Chester disease. Bone biopsy showed langerhans cell histiocytosis CD1a positive with the presence of BRAF V600E mutation. The patient was treated with vemurafenib with a good clinical course. CONCLUSION: The literature review finds forty observations linking the two diseases that may suggest a pathophysiological link, especially with the hematopoietic myeloid stem cell CD34+. The term inflammatory myeloid neoplasm was advanced.

[Diaphragm of inferior vena cava associated with Budd-Chiari syndrome. Contribution of ultrasonography and color Doppler echocardiography]. / Diaphragme de la veine cave inférieure associé à un syndrome de Budd-Chiari. Apport de l'échographie et du Doppler couleur.

We report the case of a young man presenting clinical features of portal hypertension of unknown origin. The ultrasonographic examination showed suprahepatic blockage signs leading to the diagnosis of Budd-Chiari syndrome caused by incomplete diaphragm of the inferior vena cava. The color Doppler examination suggested caval obstruction, showing an abrupt shift from a laminar to a bosterous flux. Secondly cavograms and surgery later confirmed the diagnosis.