Classical Type: Diagnostic Criteria

The major diagnostic criteria for Classical Type EDS are

The minor diagnostic criteria for Classical Type EDS may include

  • Smooth velvety skin
  • molluscoid pseudo tumors
  • Subcutaneous spheroids
  • Complications of joint hypermobility (sprains, islocations/subluxations, pes planus, etc.)
  • Muscle hypotonia, delayed gross motor development
  • Easy bruising
  • Manifestations of tissue extensibility and fragility (e.g., hiatal hernia, anal prolapse in childhood, cervical insufficiency)
  • Surgical complications (post-operative hernias)
  • Family history

Additional comments

  • The skin manifestations range in severity; families with mild, moderate and severe expression have been described.
  • Molluscoid pseudotumors are fleshy lesions associated with scars. They are frequently found over pressure points (e.g., elbows).
  • Spheroids are small subcutaneous spherical hard bodies, frequently mobile and palpable on the forearms and shins. Spheroids may be calcified and detectable radiologically.
  • Recurrent joint subluxations are frequent in the shoulder, patella, and temporomandibular joints.
  • Dyspareunia and sexual dysfunction are occasional complaints in the Classical and other types of EDS
  • Fatigue is a frequent complaint.