Ehlers-Danlos Syndrome Type VIII
نویسندگان
چکیده
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 violaceous plaques for more than 20 years. Premature loss of permanent teeth is also noted. Combining the clinical manifestations and laboratory examinations, we conclude this is a rare case of Ehlers-Danlos syndrome type VIII. There is no specific treatment currently. (Dermatol Sinica 24: 150-154, 2006)
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 کاملEhlers-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 کاملEhlers-Danlos syndrome identified from periodontal findings: case report.
The Ehlers-Danlos syndrome comprises a group of generalized connective tissue disorders characterized by fragile skin, skin hyperextensibility, and joint hypermobility. More than 10 types of Ehlers-Danlos syndrome have been identified based on genetic and biochemical studies. What is now known as Ehlers-Danlos syndrome (EDS) Type VIII was first described in 1972. We report a case of EDS Type VI...
متن کاملEhlers-Danlos Syndrome Type VIII: A Rare Cause of Leg Ulcers in Young Patients
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 ...
متن کاملAn Iranian family with cutis laxa and classic Ehlers-Danlos syndrome
Ehlers-Danlos syndrome (EDS) is a heterogenous group of inherited disorders of connective tissue characterized by fragility of the skin and blood vessels, hyperextensibility of the skin and joint hypermobility. Cutis laxa is characteized clinically by lax, pendulous skin and histologically by loss of elastic tissue in the dermis. There are some reports of coexistence of cutis laxa with ot...
متن کامل