![]() ![]() She had an accident due to sudden inversion of her left foot with subsequent fracture of both tibia and fibula with crushed left medial malleolus, which was surgically corrected. There was history of frequent subluxation and mild dislocation of her right shoulder joint associated with occasional pain since 20 years. Her first child was born at eight months due to premature rupture of the amniotic membrane. She had also suffered from sprain of her left foot on many occasions and a fracture of 5 th metratarsal bone at 23 years of age. She gave history of frequent falls since childhood, either due to sudden inversion of left foot or sudden flexion of left knee. We came across two family members of this pedigree in four generations showing a new sign, which we would like to label as ‘Reverse-Namaskar’ sign.Ī 68-year-old female scientist presented with asymptomatic multiple firm subcutaneous nodular lesions over both the upper extremities since two decades. These are subcutaneous nodules due to herniation of subcutaneous fatty tissue and resemble lipomas histologically. Molluscoid pseudo tumors and spheroids may occur in EDS. The diagnostic skin signs described in EDS includes Gorlin sign (ability to touch the tip of the nose with the tongue), Metenier sign (easy eversion of upper eyelid) and atrophic ‘cigarette paper’ scarring. In a recent consensus in Villefranche, in 1997, the classification of EDS was reorganized into six major subtypes. ![]() ![]() The phenotype varies depending upon the type of collagen altered. ![]() Cutaneous, vascular, skeletal, ocular and dental manifestations have been described. The diagnosis in classic EDS (EDS I and II) is established mainly by the family history and the characteristic clinical findings.ĮDS is caused by underlying ultra structural abnormalities in the collagen fibrils, which is genetically determined. It is a rare genetic disorder affecting 1 in 5000 people. Ehlers-Danlos syndrome (EDS) belongs to a heterogeneous group of disorders of collagen characterized by hyper extensibility and fragility of the skin with easy bruisability and hyper mobility of the joints. ![]()
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