However, joint hypermobility is similar to that Scarring is less common, bleeding tendency is less pronounced and nodules are Similar to type I, this form is less severe clinically. Over half of pregnant women with the condition give birth prematurely,įollowing rupture of the fetal membranes. Joints there is usually an abnormal bleeding tendency. Along with a generalized hypermobility of the The patient usually has a mesomorphic appearance with hands and feet being Scars (“cigarette paper scar”) are frequently found in these areas and theyĪre accompanied at times by fibrous nodules caused by the fibrous transformation Limited healing power of the skin with this condition, pigmented and atrophic Bony prominences such as theįorehead, chin, elbows, knees and ankles are constantly lacerated. 5,8 We present a description of theįirst 8 of these phenotypes, but recommend that you read the works of Pope 1 Tendency to excessive bleeding manifested by bruises, ecchymoses and hematomas.Īt least 15 phenotypes of the syndromes have been catalogued to date, with 2 Skin, which is soft, thin and fragile the presence of dystrophic scars and a The classic signs of EDS are joint hypermobility hyperelasticity of The differential diagnosis of EDS includes Marfan’s syndrome, generalizedįamilial joint hypermobility synd rome, cutis laxa, pseudoxanthoma elasticum and 7Įven when there is a bleeding disorder associated with the syndrome, bloodĪnalyses are not diagnostically useful, in that no correlation has been madeīetween the findings of such tests and the various types of EDS. Of collagen, it is especially difficult to establish a precise diagnosis. Only 4 types of EDS, namely types IV, VI, VII 6 and X 5 canīe confirmed by biochemical and molecular tests. Primarily on clinical findings and family history, as it is an autosomalĭominant hereditary disorder which presents in several ways (see Tableġ, Classification of Ehlers-Danlos syndrome, The prevalence of the condition varies between 1:10,000 5Īnd 1:150,000 6 depending on the author. Precautions to take when providing dental treatment for EDS patients. Joint (TMJ) as well as vascular manifestations inherent to the syndrome, and the The case of a 12-year-old affected by hypermobility of the temporo-mandibular Of this article is to review current knowledge about the syndrome, to present Several articles describe the skin and joint problems linked to EDS, but veryįew describe the oral manifestations of the condition. Other evocative terms such as “elastic man” (or woman) or “India rubber Pommeau-Delille and Soussie described the condition as Ehlers-Danlos syndrome. In 1908, Danlos introduced the idea that the condition representedĪ pseudo-tumour of a molluscoid or fibrous type. 1 In 1901, Ehlersĭescribed the condition as a hyperelasticity of the skin and a strong tendency Muscle over to the left angle of his mandible. The first description of the syndrome in the literature was ofĪ young Spaniard who was able to stretch the skin overlying his right pectoral Is a hereditary collagen disease presenting primarily as dermatological and MeSH Key Words: case report dental care for chronically ill Ehlers-Danlos syndrome The article also describes the case of a 12-year-old patient presenting with hypermobility of the temporo-mandibular joint and capillary fragility, and highlights the precautions to take when treating patients with this syndrome. A brief history of the disease is presented along with the epidemiology and characteristics of the 8 main phenotypes of the syndrome. Oral Manifestations of Ehlers-Danlos SyndromeĮhlers-Danlos syndrome is a rare hereditary disease of the connective tissue which can present oral manifestations.
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