References

Ferreira O, Cardoso CL, Alvares Capelozza AL, Faria Yaedu RY, da Costa AR. Odontogenic keratocyst and multiple supernumerary teeth in a patient with Ehlers-Danlos syndrome – a case report and review of the literature. Quintessence Int. 2008; 39:251-256
Gazit Y, Nahir AM, Grahame R, Jacob G. Dysautonomia in the joint hypermobility syndrome. Am J Med. 2003; 115:33-40
Pope FM. Ehlers-Danlos syndrome. Baillieres Clin Rheumatol. 1991; 5:321-349
Letourneau Y, Perusse R, Buithieu H. Oral manifestations of Ehlers-Danlos syndrome. J Can Dent Assoc. 2001; 67:330-334
Hagberg C, Berglund B, Korpe L, Andersson-Norinder J. Ehlers-Danlos Syndrome (EDS) focusing on oral symptoms: a questionnaire study. Orthod Craniofac Res. 2004; 7:178-185
Hakim AJ, Grahame R, Norris P, Hopper C. Local anaesthetic failure in joint hypermobility syndrome. J R Soc Med. 2005; 98:84-85
Abel MD, Carrasco LR. Ehlers-Danlos syndrome: classifications, oral manifestations, and dental considerations. Oral Surg Oral Med Oral Pathol Oral Radiol Endodontol. 2006; 102:582-590
Steinmann B, Royce PM, Superti-Furga A. The Ehlers-Danlos Syndrome, 2nd edn. In: Roce PM SB (ed). New York: Wiley-Liss; 2002
Castori M. Ehlers-danlos syndrome, hypermobility type: an underdiagnosed hereditary connective tissue disorder with mucocutaneous, articular, and systemic manifestations. ISRN Dermatol. 2012; 2012:751-768
Castori M, Camerota F, Celletti C, Grammatico P, Padua L. Ehlers-Danlos Syndrome Hypermobility Type and the excess of affected females: possible mechanisms and perspectives. Am J Med Genet. 2010; 152A:2406-2408
Beighton P, De Paepe A, Steinmann B, Tsipouras P, Wenstrup RJ. Ehlers-Danlos syndromes: revised nosology, Villefranche, 1997. Am J Med Genet. 1998; 77:31-37
Malfait F, Francomano C, Byers P, Belmont J, Berglund B, Black J The 2017 International Classification of the Ehlers-Danlos Syndromes. Am J Med Genet – Seminars in Medical Genetics. 2017; 175C:8-26
De Paepe A, Malfait F. Bleeding and bruising in patients with Ehlers-Danlos syndrome and other collagen vascular disorders. Br J Haematol. 2004; 127:491-500
Hakim AJ, Grahame R. A simple questionnaire to detect hypermobility: an adjunct to the assessment of patients with diffuse musculoskeletal pain. Int J Clin Pract. 2003; 57:163-166
Arendt-Nielsen lKS, Bjerring P, Hogsaa B. Insufficient effect of local analgesics in Ehlers-Danlos type 3 patients (Connective tissue disorder). Acta Anaesthesiol Scand. 1990; 34:358-361
Mitakides J, Tinkle BT. Oral and mandibular manifestations in the Ehlers-Danlos Syndromes. Am J Med Genet – Seminars in Medical Genetics. 2017; 175C:220-225
Brady AF, Demirdas S, Fournel-Gigleux S, Ghali N, Giunta C, Kapferer-Seebacher I The Ehlers-Danlos Syndromes, rare types. Am J Med Genet – Seminars in Medical Genetics. 2017; 175C:70-115
Kapferer-Seebacher I, Pepin M, Werner R, Aitman TJ, Nordgren A, Stoiber H Periodontal Ehlers-Danlos Syndrome is caused by mutations in C1R and C1S, which encode subcomponents C1r and C1s of complement. Am J Hum Genet. 2016; 99:1005-1014
Norton LA, Assael AL. Orthodontic and temperomandibular joint considerations in the treatment of patients with Ehlers-Danlos syndrome. Am J Orthod Dentofacial Orthop. 1997; 111:75-84
Jones ML. Orthodontic treatment in Ehlers-Danlos syndrome. Br J Orthod. 1984; 11:158-162
Craig DBC. Practical Conscious Sedation, 2nd edn. London: Quintessence; 2017
Beighton P. Serious ophthalmological complications in the Ehlers-Danlos syndrome. Br J Ophthalmol. 1970; 54:263-268
Gorlin RJ Syndromes of the Head and Neck.New York: Oxford University Press; 1990
Klingberg G, Hagberg C, Noren JG, Nietzsche S. Aspects on dental hard tissues in primary teeth from patients with Ehlers-Danlos syndrome. Int J Paediatr Dent. 2009; 19:282-290
Carr RJ, Green DM. Multiple odontogenic keratocysts in a patient with type-ii (mitis) Ehlers-Danlos syndrome. Br J Oral Maxillofac Surg. 1988; 26:205-214
Hakim A, Grahame R. Joint hypermobility. Best Pract Res Clin Rheumatol. 2003; 17:989-1004
Prophylaxis against infective endocarditis: antimicrobial prophylaxis against infective endocarditis in adults and children undergoing interventional procedures. NICE Guideline [CG64]. 2008;
Antibiotic Prophylaxis Against Infective Endocarditis.Dundee: SDCEP; 2018
Mathias CJ, Low DA, Iodice V, Owens AP, Kirbis M, Grahame R. Postural tachycardia syndrome-current experience and concepts. Nature Rev Neurol. 2012; 8:22-34
Morgan AW, Pearson SB, Davies S, Gooi HC, Bird HA. Asthma and airways collapse in two heritable disorders of connective tissue. Ann Rheumat Dis. 2007; 66:1369-1373
Bendik EM, Tinkle BT, Al-shuik E, Levin L, Martin A, Thaler R Joint hypermobility syndrome: a common clinical disorder associated with migraine in women. Cephalalgia. 2011; 31:603-613
Castori M, Morlino S, Celletti C, Celli M, Morrone A, Colombi M Management of pain and fatigue in the joint hypermobility syndrome (a.k.a. Ehlers-Danlos syndrome, hypermobility type): principles and proposal for a multidisciplinary approach. Am J Med Genet. 2012; 158A:2055-2070
Martin VT, Neilson D. Joint hypermobility and headache: the glue that binds the two together – Part 2. Headache. 2014; 54:1403-1411
Henderson FC, Austin C, Benzel E, Bolognese P, Ellenbogen R, Francomano CA Neurological and spinal manifestations of the Ehlers-Danlos syndromes. Am J Med Genet – Seminars in Medical Genetics. 2017; 175C:195-211
Neilson D, Martin VT. Joint hypermobility and headache: understanding the glue that binds the two together – Part 1. Headache. 2014; 54:1393-1402
Hakim A, De Wandele I, O'Callaghan C, Pocinki A, Rowe P. Chronic fatigue in Ehlers-Danlos syndrome-hypermobile type. Am J Med Genet – Seminars in Medical Genetics. 2017; 175C:175-180
Hershenfeld SA, Wasim S, McNiven V, Parikh M, Majewski P, Faghfoury H Psychiatric disorders in Ehlers-Danlos syndrome are frequent, diverse and strongly associated with pain. Rheumatol Int. 2016; 36:341-348
Seneviratne SL, Maitland A, Afrin L. Mast cell disorders in Ehlers-Danlos syndrome. Am J Med Genet – Seminars in Medical Genetics. 2017; 175C:226-236

Ehlers-Danlos syndrome: a review

From Volume 46, Issue 7, July 2019 | Pages 634-644

Authors

Samina Nayani

BDS(Hons), MFDS RCSEd

Specialty Registrar in Special Care Dentistry, King's College Hospital, Denmark Hill, London SE5 9RS, UK

Articles by Samina Nayani

Email Samina Nayani

Joanna Dick

BDS, MJDF RCSEng, DipDSed

Specialty Registrar in Special Care Dentistry, The Royal London Dental Hospital, Turner Street, Whitechapel, London E1 1BB

Articles by Joanna Dick

Charlotte Curl

BDS(Hons), FDS RCS(Eng) DSCD RCS(Eng), PgDipClinEd FHEA

Senior Dental Officer, Dental Department, Hainault Health Centre, Manford Way, Chigwell, Essex IG7 4DF

Articles by Charlotte Curl

Abstract

Ehlers-Danlos Syndrome (EDS) affects the metabolism of collagen which can have implications throughout the body, impacting on not only the skin, but also the joints, muscles, cardiovascular and gastrointestinal systems. The condition can have dental implications such as poor wound healing, mucosal fragility, prolonged bleeding, temporomandibular joint dislocation and, in some forms, periodontal disease. Three clinical cases of patients with EDS will be discussed and their dental management described. The patient with EDS may be seen in primary care and, if input from secondary care is required, a multidisciplinary, shared care approach will ideally be utilized.

CPD/Clinical Relevance: Ehlers-Danlos Syndrome is a condition that can be multifactorial, with medical implications as well as dental; depending on the manifestations of the condition, the patient may be seen in primary and/or secondary care with or without a shared care approach.

Article

Ehlers Danlos Syndrome (EDS) is a rare syndrome characterized by 13 types of connective tissue disorders which manifest primarily in dermatological and joint conditions. EDS affects collagen metabolism, leading to deficiency and/or disordered deposition of collagen.1 Within the literature there is an overlap between the EDS subtypes and other connective tissue diseases, including hypermobility disorders, for example Joint Hypermobility Syndrome (JHS), which is often referred to interchangeably with EDS.2

The condition was first described in 1657 when a young Spanish man was found to be able to stretch the skin overlying his right pectoral muscle over the left angle of his mandible.3 Further descriptors were added in 1901 by Ehlers, 1908 by Danlos, and then by Poumeau-Delille and Soulie in 1934 when the condition was described as Ehlers-Danlos Syndrome.4

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