Danlos syndrome
Better known today as Ehlers-Danlos syndrome (EDS), this is an inherited disorder with easy bruising, joint hypermobility (loose joints), skin laxity, and weakness of tissues. There are a number of different Ehlers-Danlos syndromes which share these features but can be categorized into nine different types. Type I EDS (the “gravis” form od EDS) is characterized by marked joint hypermobility, skin hyperextensibility (laxity), and fragility. Joint dislocations and scoliosis. It is inherited as an autosomal (non-sex-linked) dominant genetic trait. Dominant means that a single gene is capable of producing the disease. Type II EDS (the “mitis” form of EDS) is similar to type I, but is less severe. It, too, is inherited as an autosomal dominant genetic trait. Type III EDS (the benign hypermobility form of EDS). Joint hypermobility is the major manifestation. It is inherited as an autosomal dominant genetic trait. Type IV EDS (the arterial form) Spontaneous rupture of arteries and bowel is a serious manifestation. Skin laxity is variable. It is inherited as an autosomal dominant and recessive genetic trait. Type V EDS is clinically similar to type II but is X-linked. The gene is on the X chromosome). If a woman is carrying the gene, the chance for each of her children her son to receive the gene is 50:50. A son with the gene is affected with the disease while a daughter with the gene is merely a carrier like her mother. Type VI EDS (the ocular-scoliotic form of EDS) is characterized by a fragile globe of the eyes, significant skin and joint laxity, and severe curvature of the spine (scoliosis). It is inherited as an autosomal (non-sex-linked) recessive genetic trait. Recessive means that two copies of the gene are required to produce the disease. Type VII EDS (arthrochalasis multiplex congenita). Patients with this type of EDS are short in height and severely affected by joint laxity and dislocations. Skin involvement is variable. Autosomal dominant and recessive inheritance is possible. Type VIII EDS. Patients have different degrees of joint hypermobility and inflammation of the gums and bone adjacent to the teeth (periodontitis). Type IX EDS. Patients have mildly hypermobile joints and can have mitral valve prolapse. It is inherited as autosomal dominant.

Medical dictionary. 2011.

Look at other dictionaries:

  • Ehlers–Danlos syndrome — Classification and external resources The collagen fibril and EDS. (a) Normal collagen fibrils are of uniform size and spacing. Fibrils from a patient with dermatosparaxis (b) show dramatic alterations in fibril morphology with severe effects on… …   Wikipedia

  • Ehlers-Danlos syndrome — Infobox Disease | Name = Ehlers Danlos syndrome | ICD10 = ICD10|Q|79|6|q|65 | ICD9 = ICD9|756.83 | OMIM = | MedlinePlus = 001468 eMedicineSubj = derm eMedicineTopic = 696 eMedicine mult = eMedicine2|ped|654 | MeshID = D004535 Ehlers Danlos… …   Wikipedia

  • Ehlers-Danlos syndrome — A heritable disorder of connective tissue with easy bruising, joint hypermobility (loose joints), skin laxity, and weakness of tissues. There are a number of different types of Ehlers Danlos syndrome (EDS) which share the foregoing features but… …   Medical dictionary

  • Ehlers-Danlos syndrome — Ehlers Danlos syndrome. См. синдром Элерса Данло. (Источник: «Англо русский толковый словарь генетических терминов». Арефьев В.А., Лисовенко Л.А., Москва: Изд во ВНИРО, 1995 г.) …   Молекулярная биология и генетика. Толковый словарь.

  • ehlers-danlos syndrome — ˈālərzˈdan(ˌ)läs noun Usage: usually capitalized E&D Etymology: after E. Ehlers died 1937 Dan. dermatologist and H.A. Danlos died 1912 French dermatologist : an inherited disorder of connective tissue with several clinical forms characterized… …   Useful english dictionary

  • Ehlers-Danlos syndrome — /ay leuhrz dan los, leuhs/, Pathol. a rare hereditary disease of connective tissue, characterized by joint hypermotility and abnormally stretchable skin. [after Edvard Ehlers (1863 1937), Danish dermatologist, and Henri Alexandre Danlos (1844… …   Universalium

  • Ehlers-Danlos syndrome — any one of a rare group of inherited (autosomal dominant or autosomal recessive) disorders of the connective tissue involving abnormal or deficient collagen, the protein that gives the body tissues strength. There are several types of differing… …   The new mediacal dictionary

  • Ehlers-Danlos syndrome — See dermatosparaxis …   Dictionary of molecular biology

  • Syndrome d'Ehlers-Danlos type vasculaire — Pour les articles homonymes, voir Syndrome d Ehlers Danlos. Le syndrome d Ehlers Danlos type vasculaire est, dans le groupe des syndromes d Ehlers Danlos, caractérisé par une peau fine, translucide, se lésant facilement; une apparence du visage… …   Wikipédia en Français

  • Syndrome, Danlos — Better known today as Ehlers Danlos syndrome (EDS), is an inherited disorder with easy bruising, joint hypermobility (loose joints), skin laxity, and weakness of tissues. There are a number of different Ehlers Danlos syndromes which share these… …   Medical dictionary

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