eMedicine Specialties > Dermatology > Pediatric Diseases

Ehlers-Danlos Syndrome: Treatment & Medication

Author: Enrico Ceccolini, MD, Consulting Staff, Department of Dermatology, University of Bologna, Italy
Coauthor(s): Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Contributor Information and Disclosures

Updated: Feb 18, 2009

Treatment

Medical Care

  • Treatment is unsatisfactory.
  • One isolated report showed that patients with type VI disease benefited from oral vitamin C at 4 g/d. Scars and bleeding time seemed to improve with this treatment.

Surgical Care

  • Extreme caution is mandatory in any surgical maneuver.
  • Plastic re-excision of scars sometimes provides acceptable cosmetic results.
  • Anesthetic implications, although rare, are very important in Ehlers-Danlos syndrome, especially in the type IV (or vascular) patients. Risk of complications is higher, spontaneous vascular rupture can occur, and cervical spine and airway trauma must be kept in mind. Bleeding is also reported.22
  • Recombinant factor VIIa has been successful in the treatment of intractable bleeding in vascular-type Ehlers-Danlos syndrome.23

Activity

  • Patients with Ehlers-Danlos syndrome types IV or VI should avoid participating in dangerous contact sports.
  • Some authors mention risks with activities that can increase intracranial pressure as a result of the Valsalva effect. An example of one such activity is playing the trumpet.

Medication

The goals of pharmacotherapy are to prevent complications and reduce morbidity.

Vitamins

Vitamin C may improve morbidity. It is a critical cofactor for collagen fibril synthesis.


Ascorbic acid (Cecon, Cevalin, Cevi-Bid)

For collagen synthesis and tissue repair.

Adult

100-250 mg PO qd/bid for at least 2 wk

Pediatric

100-300 mg PO in divided doses for at least 2 wk

Decreases effects of warfarin and fluphenazine; increases aspirin levels

Pregnancy

A - Fetal risk not revealed in controlled studies in humans

Precautions

Prolonged high doses may cause renal calculi, especially in diabetes

More on Ehlers-Danlos Syndrome

Overview: Ehlers-Danlos Syndrome
Differential Diagnoses & Workup: Ehlers-Danlos Syndrome
Treatment & Medication: Ehlers-Danlos Syndrome
Follow-up: Ehlers-Danlos Syndrome
Multimedia: Ehlers-Danlos Syndrome
References
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References

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Further Reading

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Keywords

Ehlers-Danlos syndrome, EDS, tenascin-X deficiency syndrome, lysyl hydroxylase deficiency syndrome, inherited connective tissue disorders, inherited connective-tissue disorders, connective-tissue disorder, connective tissue disorder

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Contributor Information and Disclosures

Author

Enrico Ceccolini, MD, Consulting Staff, Department of Dermatology, University of Bologna, Italy
Enrico Ceccolini, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

Coauthor(s)

Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi
Disclosure: Nothing to disclose.

Medical Editor

Noah S Scheinfeld, MD, JD, FAAD, Assistant Clinical Professor, Department of Dermatology, Columbia University; Consulting Staff, Department of Dermatology, St Luke's Roosevelt Hospital Center, Beth Israel Medical Center, New York Eye and Ear Infirmary; Private Practice
Noah S Scheinfeld, MD, JD, FAAD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Optigenex Consulting fee Independent contractor

Pharmacy Editor

David F Butler, MD, Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic
David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa
Disclosure: Nothing to disclose.

Managing Editor

Warren R Heymann, MD, Head, Division of Dermatology, Professor, Department of Internal Medicine, University of Medicine and Dentistry of New Jersey
Warren R Heymann, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

CME Editor

Glen H Crawford, MD, Assistant Clinical Professor, Department of Dermatology, University of Pennsylvania School of Medicine; Chief, Division of Dermatology, The Pennsylvania Hospital
Glen H Crawford, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Phi Beta Kappa, and Society of USAF Flight Surgeons
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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