title: Two case presentations of profound labial edema as a presenting symptom of hypermobility-type Ehlers-Danlos syndrome.
abstract: BACKGROUND
  Hypermobility-type Ehlers-Danlos syndrome (EDS), an often-missed diagnosis with the potential for serious sequelae, may have a variety of uncommon presentations, some of which may be gynecologic.
  
  
  OBJECTIVE
  The aim of this case report is to present two cases of profound labial edema associated with intercourse as a presenting symptom of hypermobility-type EDS.
  
  
  METHODS
  A 25-year-old female presented with severe labia minora swelling and bladder pressure associated with intercourse, in addition to persistent genital arousal. History revealed easy bruising, joint pain, and family history of aneurysm. A 22-year-old female presented with intermittent profound labial swelling for 6 years, associated with sensitivity and pain with intercourse. The patient has a history of joint pain and easy bruising, as well a sister with joint hypermobility and unexplained lymphedema. The presenting symptom of profound labial edema led to the diagnosis of hypermobility-type EDS.
  
  
  RESULTS
  Patients with hypermobility syndrome exhibit an increased ratio of type III collagen to type I collagen, causing tissue laxity and venous insufficiency. Abnormal collagen may lead to gynecologic manifestations, including unexplained profound labial edema, pelvic organ prolapse in the absence of risk factors, and possibly persistent genital arousal.
  
  
  CONCLUSIONS
  This case report highlights the need for further research to determine incidence of labial edema in hypermobility-type EDS and to further elucidate a potential correlation between profound labial edema and collagen disorders.
authors.affiliation: Department of Obstetrics and Gynecology, The George Washington University, Washington, DC, USA.
authors.forename: Jill M
authors.initials: JM
authors.lastname: Krapf
authors.forename: Andrew T
authors.initials: AT
authors.lastname: Goldstein
mesh_terms: D000328:Adult; D001143:Arousal; D003075:Coitus; D004414:Dyspareunia / Q000209:etiology; D004487:Edema / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology; D004535:Ehlers-Danlos Syndrome / Q000150:complications* / Q000175:diagnosis / Q000503:physiopathology; D005260:Female; D005820:Genetic Testing; D006801:Humans; D009948:Orgasm; D011237:Predictive Value of Tests; D020018:Sexual Dysfunctions, Psychological / Q000209:etiology; D014845:Vulvar Diseases / Q000175:diagnosis / Q000209:etiology* / Q000503:physiopathology; D055815:Young Adult
keywords: Ehlers-Danlos syndrome; Labial Edema; Persistent Genital Arousal Disorder