title: [Generalized pseudoxanthoma elasticum combined with vitamin K dependent clotting factors deficiency].
abstract: BACKGROUND
  Pseudoxanthoma elasticum is a connective tissue disease currently classed in 4 forms. Two forms are inherited via dominant autosomal transmission and the other two via recessive autosomal transmission. The generalized form of pseudoxanthoma elasticum is the most uncommon form and corresponds to recessive type II. Clinical manifestations include the typical generalized "peau d'orange" skin associated with hyperlaxity of the skin. Usually, there is no systemic manifestation.
  
  
  METHODS
  We report a case of a patient with generalized pseudoxanthoma elasticum associated with deficiency of vitamin-K dependent factors II, VII, IX, and X. A search for other causes of vitamin-K dependent factor deficiency was negative.
  
  
  CONCLUSIONS
  The association of generalized pseudoxanthoma elasticum with deficiency of vitamin-K dependent clotting factors has been reported previously in very rare cases and is probably not fortuitous. It could led to the definition of a sub-group of recessive autosomal pseudoxanthoma elasticum.
authors.affiliation: Clinique Dermatologique, Hôpital Charles-Nicolle, Rouen.
authors.forename: C
authors.initials: C
authors.lastname: Le Corvaisier-Pieto
authors.forename: P
authors.initials: P
authors.lastname: Joly
authors.forename: E
authors.initials: E
authors.lastname: Thomine
authors.forename: G
authors.initials: G
authors.lastname: Lair
authors.forename: P
authors.initials: P
authors.lastname: Lauret
mesh_terms: D000328:Adult; D001778:Blood Coagulation Disorders / Q000097:blood*; D005168:Factor VII Deficiency / Q000150:complications; D005171:Factor X Deficiency / Q000150:complications; D006801:Humans; D007020:Hypoprothrombinemias / Q000097:blood*; D008297:Male; D011517:Prothrombin Time; D011561:Pseudoxanthoma Elasticum / Q000150:complications* / Q000235:genetics / Q000473:pathology; D014813:Vitamin K Deficiency / Q000150:complications* / Q000235:genetics
vernacular_title: Pseudoxanthome élastique généralisé associé à un déficit en facteurs vitamine-K dépendants.