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Ehlers-Danlos Syndrome Medication

  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD  more...
Updated: Jun 09, 2016

Medication Summary

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



Class Summary

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

Ascorbic acid (Cecon, Cevalin, Cevi-Bid)


Ascorbic acid helps with collagen synthesis and tissue repair.

Contributor Information and Disclosures

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public Affairs and Administration

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, New York Academy of Medicine, American Academy of Dermatology, American College of Physicians, Sigma Xi

Disclosure: Nothing to disclose.


Enrico Ceccolini, MD Consulting Staff, Department of Dermatology, University of Bologna, Italy. Private Practice, Pesaro, Italy.

Enrico Ceccolini, MD is a member of the following medical societies: American Academy of Dermatology, International Society of Dermatology, Society for Pediatric Dermatology

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

David F Butler, MD is a member of the following medical societies: American Medical Association, Alpha Omega Alpha, Association of Military Dermatologists, American Academy of Dermatology, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Phi Beta Kappa

Disclosure: Nothing to disclose.

Warren R Heymann, MD Head, Division of Dermatology, Professor, Department of Internal Medicine, Rutgers New Jersey Medical School

Warren R Heymann, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, Society for Investigative Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Noah S Scheinfeld, JD, MD, FAAD Assistant Clinical Professor, Department of Dermatology, Weil Cornell Medical College; Consulting Staff, Department of Dermatology, St Luke's Roosevelt Hospital Center, Beth Israel Medical Center, New York Eye and Ear Infirmary; Assistant Attending Dermatologist, New York Presbyterian Hospital; Assistant Attending Dermatologist, Lenox Hill Hospital, North Shore-LIJ Health System; Private Practice

Noah S Scheinfeld, JD, MD, FAAD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Serve(d) as a speaker or a member of a speakers bureau for: Abbvie<br/>Received income in an amount equal to or greater than $250 from: Optigenex<br/>Received salary from Optigenex for employment.

  1. Eder J, Laccone F, Rohrbach M, Giunta C, Aumayr K, Reichel C, et al. A new COL3A1 mutation in Ehlers-Danlos syndrome type IV. Exp Dermatol. 2013 Mar. 22 (3):231-4. [Medline].

  2. Schwarze U, Atkinson M, Hoffman GG, Greenspan DS, Byers PH. Null alleles of the COL5A1 gene of type V collagen are a cause of the classical forms of Ehlers-Danlos syndrome (types I and II). Am J Hum Genet. 2000 Jun. 66 (6):1757-65. [Medline].

  3. Bouma P, Cabral WA, Cole WG, Marini JC. COL5A1 exon 14 splice acceptor mutation causes a functional null allele, haploinsufficiency of alpha 1(V) and abnormal heterotypic interstitial fibrils in Ehlers-Danlos syndrome II. J Biol Chem. 2001 Apr 20. 276 (16):13356-64. [Medline].

  4. Wenstrup RJ, Florer JB, Willing MC, Giunta C, Steinmann B, Young F, et al. COL5A1 haploinsufficiency is a common molecular mechanism underlying the classical form of EDS. Am J Hum Genet. 2000 Jun. 66 (6):1766-76. [Medline].

  5. Sun M, Connizzo BK, Adams SM, Freedman BR, Wenstrup RJ, Soslowsky LJ, et al. Targeted deletion of collagen V in tendons and ligaments results in a classic Ehlers-Danlos syndrome joint phenotype. Am J Pathol. 2015 May. 185 (5):1436-47. [Medline].

  6. Yeowell HN, Walker LC. Mutations in the lysyl hydroxylase 1 gene that result in enzyme deficiency and the clinical phenotype of Ehlers-Danlos syndrome type VI. Mol Genet Metab. 2000 Sep-Oct. 71 (1-2):212-24. [Medline].

  7. Schalkwijk J, Zweers MC, Steijlen PM, Dean WB, Taylor G, van Vlijmen IM, et al. A recessive form of the Ehlers-Danlos syndrome caused by tenascin-X deficiency. N Engl J Med. 2001 Oct 18. 345 (16):1167-75. [Medline].

  8. Boudko SP, Engel J, Okuyama K, Mizuno K, Bächinger HP, Schumacher MA. Crystal structure of human type III collagen Gly991-Gly1032 cystine knot-containing peptide shows both 7/2 and 10/3 triple helical symmetries. J Biol Chem. 2008 Nov 21. 283 (47):32580-9. [Medline].

  9. Okamoto O, Ando T, Watanabe A, Sato F, Mimata H, Shimada T, et al. A novel point mutation in type III collagen gene resulting in exon 24 skipping in a case of vascular type Ehlers-Danlos syndrome. Arch Dermatol Res. 2008 Oct. 300 (9):525-9. [Medline].

  10. Viglio S, Zoppi N, Sangalli A, Gallanti A, Barlati S, Mottes M, et al. Wound repair capability in EDS fibroblasts can be retrieved by exogenous type V collagen. ScientificWorldJournal. 2008 Oct 3. 8:956-8. [Medline].

  11. Kuz'mina NS, Shipaeva EV, Semyachkina AN, Vasil'eva IM, Kovalenko LP, Durnev AD, et al. Polymorphism of detoxification genes and cell resistance to mutagens in patients with Ehlers-Danlos syndrome. Bull Exp Biol Med. 2007 Nov. 144 (5):717-21. [Medline].

  12. Murray ML, Yang M, Fauth C, Byers PH. FKBP14-related Ehlers-Danlos syndrome: expansion of the phenotype to include vascular complications. Am J Med Genet A. 2014 Jul. 164A (7):1750-5. [Medline].

  13. Bethea BT, Fitton TP, Alejo DE, Barreiro CJ, Cattaneo SM, Dietz HC, et al. Results of aortic valve-sparing operations: experience with remodeling and reimplantation procedures in 65 patients. Ann Thorac Surg. 2004 Sep. 78 (3):767-72; discussion 767-72. [Medline].

  14. Remvig L, Flycht L, Christensen KB, Juul-Kristensen B. Lack of consensus on tests and criteria for generalized joint hypermobility, Ehlers-Danlos syndrome: hypermobile type and joint hypermobility syndrome. Am J Med Genet A. 2014 Mar. 164A (3):591-6. [Medline].

  15. Castori M, Dordoni C, Morlino S, Sperduti I, Ritelli M, Valiante M, et al. Spectrum of mucocutaneous manifestations in 277 patients with joint hypermobility syndrome/Ehlers-Danlos syndrome, hypermobility type. Am J Med Genet C Semin Med Genet. 2015 Mar. 169C (1):43-53. [Medline].

  16. Yassin OM, Rihani FB. Multiple developmental dental anomalies and hypermobility type Ehlers-Danlos syndrome. J Clin Pediatr Dent. 2006 Summer. 30 (4):337-41. [Medline].

  17. van Bon AC, Kristinsson JO, van Krieken JH, Wanten GJ. Concurrent splenic peliosis and vascular Ehlers-Danlos syndrome. Ann Vasc Surg. 2009 Mar. 23 (2):256.e1-4. [Medline].

  18. Guala A, Viglio S, Ottinetti A, Angeli G, Canova G, Colombo E, et al. Cutaneous metaplastic synovial cyst in Ehlers-Danlos syndrome: report of a second case. Am J Dermatopathol. 2008 Feb. 30 (1):59-61. [Medline].

  19. Vilisaar J, Harikrishnan S, Suri M, Constantinescu CS. Ehlers-Danlos syndrome and multiple sclerosis: a possible association. Mult Scler. 2008 May. 14 (4):567-70. [Medline].

  20. Perrinaud A, Matos M, Maruani A, Mondon K, Machet L. [Absence of inferior labial or lingual frenula in Ehlers-Danlos syndrome: a new diagnostic criterion?]. Ann Dermatol Venereol. 2007 Nov. 134 (11):859-62. [Medline].

  21. Baba CS, Sharma PK, Deo V, Pal S, Sethuraman G, Gupta SD, et al. Association of Ehlers-Danlos syndrome and solitary rectal ulcer syndrome. Indian J Gastroenterol. 2007 May-Jun. 26 (3):149-50. [Medline].

  22. Savasta S, Crispino M, Valli M, Calligaro A, Zambelloni C, Poggiani C. Subependymal periventricular heterotopias in a patient with ehlers-danlos syndrome: a new case. J Child Neurol. 2007 Mar. 22 (3):317-20. [Medline].

  23. Miles SC, Robinson PD, Miles JL. Ehlers–Danlos syndrome and anorexia nervosa: a dangerous combination?. Pediatr Dermatol. 2007 May-Jun. 24 (3):E1-4. [Medline].

  24. Kundu AK, Chattopadhyay P, Kundu S, Choudhury S. Pregnancy in Ehlers-Danlos syndrome. J Assoc Physicians India. 2006 Dec. 54:938. [Medline].

  25. Atzinger CL, Meyer RA, Khoury PR, Gao Z, Tinkle BT. Cross-sectional and longitudinal assessment of aortic root dilation and valvular anomalies in hypermobile and classic Ehlers-Danlos syndrome. J Pediatr. 2011 May. 158 (5):826-830.e1. [Medline].

  26. Dordoni C, Ritelli M, Venturini M, Chiarelli N, Pezzani L, Vascellaro A, et al. Recurring and generalized visceroptosis in Ehlers-Danlos syndrome hypermobility type. Am J Med Genet A. 2013 May. 161A (5):1143-7. [Medline].

  27. Hamonet C, Frédy D, Lefèvre JH, Bourgeois-Gironde S, Zeitoun JD. Brain injury unmasking Ehlers-Danlos syndromes after trauma: the fiber print. Orphanet J Rare Dis. 2016 Apr 22. 11:45. [Medline].

  28. Aalberts JJ, van den Berg MP, Bergman JE, du Marchie Sarvaas GJ, Post JG, van Unen H, et al. The many faces of aggressive aortic pathology: Loeys-Dietz syndrome. Neth Heart J. 2008 Sep. 16 (9):299-304. [Medline].

  29. Castori M. Ehlers-Danlos syndrome(s) mimicking child abuse: Is there an impact on clinical practice?. Am J Med Genet A. 2016 May 5. [Medline].

  30. Umekoji A, Fukai K, Hosomi N, Ishii M, Tanaka A, Murakami K, et al. Vascular type of Ehlers-Danlos syndrome associated with mild haemophilia A. Clin Exp Dermatol. 2009 Jan. 34 (1):101. [Medline].

  31. Rombaut L, Malfait F, De Wandele I, Cools A, Thijs Y, De Paepe A, et al. Medication, surgery, and physiotherapy among patients with the hypermobility type of Ehlers-Danlos syndrome. Arch Phys Med Rehabil. 2011 Jul. 92 (7):1106-12. [Medline].

  32. Alkadhi H, Wildermuth S, Desbiolles L, Schertler T, Crook D, Marincek B, et al. Vascular emergencies of the thorax after blunt and iatrogenic trauma: multi-detector row CT and three-dimensional imaging. Radiographics. 2004 Sep-Oct. 24 (5):1239-55. [Medline].

  33. Monroe GR, Harakalova M, van der Crabben SN, Majoor-Krakauer D, Bertoli-Avella AM, Moll FL, et al. Familial Ehlers-Danlos syndrome with lethal arterial events caused by a mutation in COL5A1. Am J Med Genet A. 2015 Jun. 167 (6):1196-203. [Medline].

  34. Fernández-Alcantud J, López RC, Osado IR, Castro MR. [Management of anesthesia in vascular-type Ehlers-Danlos syndrome (type IV)]. Rev Esp Anestesiol Reanim. 2008 May. 55 (5):312-3. [Medline].

  35. Jones TL, Ng C. Anaesthesia for caesarean section in a patient with Ehlers-Danlos syndrome associated with postural orthostatic tachycardia syndrome. Int J Obstet Anesth. 2008 Oct. 17 (4):365-9. [Medline].

  36. Faber P, Craig WL, Duncan JL, Holliday K. The successful use of recombinant factor VIIa in a patient with vascular-type Ehlers-Danlos syndrome. Acta Anaesthesiol Scand. 2007 Oct. 51 (9):1277-9. [Medline].

  37. Sinibaldi L, Ursini G, Castori M. Psychopathological manifestations of joint hypermobility and joint hypermobility syndrome/ Ehlers-Danlos syndrome, hypermobility type: The link between connective tissue and psychological distress revised. Am J Med Genet C Semin Med Genet. 2015 Mar. 169C (1):97-106. [Medline].

Patient with Ehlers-Danlos syndrome. Note the abnormal ability to elevate the right toe. Courtesy of Enrico Ceccolini, MD.
Girl with Ehlers-Danlos syndrome. Dorsiflexion of all the fingers is easy and absolutely painless. Courtesy of Enrico Ceccolini, MD.
Patient with Ehlers-Danlos syndrome mitis. Joint hypermobility is less intense than with other conditions. Courtesy of Enrico Ceccolini, MD.
Dorsal view of a patient with Ehlers-Danlos syndrome. Note the S-curved spinal column. Courtesy of Enrico Ceccolini, MD.
Cigarette-paper–like scars over the knees of a patient with Ehlers-Danlos syndrome. Note also the deformity of the left knee. Courtesy of Enrico Ceccolini, MD.
Criteria for Ehlers-Danlos syndrome are shown in Media Files 6-11. Dorsiflexion of the little finger by more than 90°with the forearm flat on the table.
Passive apposition of the thumb to the flexor forearm.
Hyperextension of the elbow by more than 90°.
Hyperextension of the knee by more than 10°.
Forward flexion of the trunk until the palms of the hands rest easily on the floor.
Evaluation of skin extensibility.
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