Ehlers-Danlos Syndrome Type VIII: A Rare Cause of Leg Ulcers in Young Patients

نویسندگان

  • Sophie Ronceray
  • Juliette Miquel
  • Antoine Lucas
  • Gérald E. Piérard
  • Trinh Hermanns-Lê
  • Anne De Paepe
  • Alain Dupuy
چکیده

Ehlers-Danlos syndrome type VIII (EDS-VIII) is a very rare autosomal dominant disease characterized by early-onset periodontitis associated with features of Ehlers-Danlos syndrome. We report a 32-year-old man whose chronic leg ulcer led to the diagnosis of EDS-VIII. He had severe periodontitis with complete loss of permanent teeth and skin fragility with thin skin, atrophic scars, and brownish atrophic pretibial plaques. Leg ulcer is not a prominent feature of EDS-VIII. We suggest adding EDS-VIII to the list of rare diseases accounting for chronic leg ulcers, if this case report prompts others to report leg ulcers associated with EDS-VIII.

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Ehlers-Danlos syndrome: type VI A – kyphoscoliosis; a case report

Ehlers-Danlos syndrome (EDS) is a clinically heterogeneous groupof inherited disorders with ten different types, all involving agenetic defect in collagen and connective-tissue synthesis andstructure that affecting the skin, joints, and blood vessels. EDStype VIA, a very rare kyphoscoliotic type, is autosomal recessiveand clinically characterized by soft extensible skin, laxity ofjoints and kyp...

متن کامل

Ehlers-Danlos syndrome type VIII with severe periodontitis and apical root resorption after orthodontic treatment.

Sir, Ehlers-Danlos syndrome (EDS) is a group of generalized disorders characterized by abnormalities of the connective tissue leading to fragility of the skin and blood vessels, hyperextensibility of the skin and joint hypermobility (1). There are many subtypes and variants of EDS; at least 10 types have been described on the basis of clinical symptoms and inheritance pattern. However, about ha...

متن کامل

Ehlers-Danlos Syndrome (TYPEI) with Mental Retardation -an Unusual Association (Reports on Two Brothers)

Ehlers Danlos syndrome (EDS) is an inherited connective tissue disease due to impaired collagen metabolism. Joint hypermobility and skin hyper extensibility are the major findings. Six types of EDS are recognized. Type I or Gravis type is characterized by skin hyperextensibility, joint hypermobility, skin splitting autosoml dominancy inheritance, preterm premature rupture of membrane (PPROM) an...

متن کامل

Ehlers-Danlos Syndrome Type VIII

Ehlers-Danlos syndrome (EDS) is a genetically heterogeneous connective tissue disorder which is comprised of more than 10 phenotypes including EDS-VIII (periodontitis type), which is characterized by chronically inflamed pretibial lesions and severe periodontitis. We describe a 26-year-old female with a long-standing history of abnormal scarring tissues, presenting with pretibial waxy violaceou...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

عنوان ژورنال:

دوره 2013  شماره 

صفحات  -

تاریخ انتشار 2013