Acquired von Willebrand syndrome (AVWS) is an increasingly recognized entity with numerous potential underlying etiologies. Most commonly implicated are lymphoproliferative, myeloproliferative, cardiovascular, and autoimmune disorders. Unlike inherited von Willebrand disease (vWD), AVWS tends to present at an older age and without a family history of vWD. Treatment is directed at the underlying...

Paraneoplastic pemphigus is an autoimmune blistering and erosive mucocutaneous syndrome associated with underlying neoplasm. It is primarily associated with lymphoproliferative disorders, and uncommonly with malignancies of epithelial origin. We report on a case of a 68-year-old male who presented with whole body bullous and erosive skin lesions. Findings on upper gastrointestinal endoscopy and...

Bénédicte Neven,1-3 *Aude Magerus-Chatinet,1,3 *Benoit Florkin,4 Delphine Gobert,1,3 Olivier Lambotte,5 Lien De Somer,6 Nina Lanzarotti,1 Marie-Claude Stolzenberg,1 Brigitte Bader-Meunier,1,2 Nathalie Aladjidi,7 Christophe Chantrain,8 Yves Bertrand,9 Eric Jeziorski,10 Guy Leverger,11 Gérard Michel,12 Felipe Suarez,13 Eric Oksenhendler,14 Olivier Hermine,3,13 Stéphane Blanche,2 Capucine Picard,2...

Heterozygous mutations in the CD95 (APO-1/Fas) receptor occur in most individuals with autoimmune lymphoproliferative syndrome (ALPS) and dominantly interfere with apoptosis by an unknown mechanism. We show that local or global alterations in the structure of the cytoplasmic death domain from nine independent ALPS CD95 death-domain mutations result in a failure to bind the FADD/MORT1 signaling ...