title: An unexpected etiology of priapism: infection-related anti-protein s antibodies.
abstract: BACKGROUND
  In adolescents, the occurrence of priapism is commonly related to sickle cell disease and rarely to other causes. We hereby report a case of priapism due to an acquired protein S (PS) deficiency.
  
  
  OBJECTIVE
  The aim of this study was to describe a young man who developed a priapism with a thrombosis of the corpora cavernosa associated with an anti-PS antibody (anti-PS Ab).
  
  
  METHODS
  One week after the onset of an influenza-like illness, a young male developed multiple extensive venous thromboses including a thrombosis of the corpora cavernosa causing painful partial priapism. These thromboses along with purpuric lesions with necrotic vesicles of the feet skin were linked to an acquired PS deficiency due to an anti-PS Ab. The optimal treatment of anti-PS Ab-associated thrombosis is debated but we chose to initiate (i) heparin; (ii) corticosteroids; and (iii) plasmapheresis.
  
  
  RESULTS
  Even if priapism lasted more than 4 days, a full recovery of erectile function was observed within 3 months. As compared with priapism due to sickle cell disease, which is commonly associated with definitive erectile dysfunction, this favorable outcome is noteworthy. The skin healing was complete only 6 months later.
  
  
  CONCLUSIONS
  Acquired PS deficiency complicating an infectious disease is a rare, life-threatening condition, associated with substantial morbidity related to amputations of limbs or digits. This is the first report of priapism due to acquired PS deficiency.
authors.affiliation: Laboratoire d'Hématologie, Centre Hospitalier Universitaire de Nantes, Nantes, France.
authors.forename: Elodie
authors.initials: E
authors.lastname: Boissier
authors.forename: Cécile
authors.initials: C
authors.lastname: Durant
authors.forename: Sophie
authors.initials: S
authors.lastname: Vildy
authors.forename: Pascal
authors.initials: P
authors.lastname: Glemain
authors.forename: Karim
authors.initials: K
authors.lastname: Lakhal
authors.forename: Julie
authors.initials: J
authors.lastname: Graveleau
authors.forename: Agathe
authors.initials: A
authors.lastname: Masseau
authors.forename: Marc
authors.initials: M
authors.lastname: Fouassier
mesh_terms: D000293:Adolescent; D001323:Autoantibodies / Q000009:adverse effects*; D006801:Humans; D008297:Male; D010413:Penis / Q000503:physiopathology; D011317:Priapism / Q000209:etiology*; D017293:Protein S / Q000276:immunology*; D018455:Protein S Deficiency / Q000150:complications* / Q000503:physiopathology
chemical_list: D001323:Autoantibodies; D017293:Protein S
keywords: Acquired Protein S Deficiency; Anti-Protein S Antibodies; Corpora Cavernosa; Ischemic Low Flow Priapism; Priapism; Thrombosis