Inside Collection (Textbook): Images of Memorable Cases: 50 Years at the Bedside
This 40-year-old woman had easy bruisability since childhood. She also had an inguinal herniorrhaphy as a teenager. An older brother died of massive gastrointestinal hemorrhage.
A group of heritable disorders of connective tissue, most of which are autosomal dominant. Characteristic manifestations are easily stretchable skin, spontaneous bleeding, and hypermobile joints. The skin may be soft, thin, and hyperelastic. Scars typically are thin and wrinkly, with a “cigarette paper” quality (left image). Sometimes, however, they appear as small molluscous pseudotumors (right image). In some cases, spontaneous arterial rupture occurs and can result in torrential bleeding from the gastrointestinal or urinary tract, tracheobronchial tree, or a gravid uterus. Articular hypermobility sometimes allows extraordinary contortionism.