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Rheumatology Practice Test
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Which combination best describes Ehlers-Danlos Syndromes?
Marfan syndrome
Scleroderma
Skin hyperextensibility, joint hypermobility, tissue fragility, vascular involvement
Osteogenesis imperfecta
Reveal Answer
Correct Answer:
Skin hyperextensibility, joint hypermobility, tissue fragility, vascular involvement
Explanation:
The main idea is recognizing the characteristic pattern of Ehlers-Danlos syndromes: a heritable connective tissue disorder where skin is unusually stretchable, joints move beyond normal range, and tissues are fragile, with vascular complications possible. This combination—skin that can be hyperextensible, joint hypermobility, tissue fragility, and potential vascular involvement—best fits EDS because it captures the hallmark cluster of manifestations seen across many EDS types. Other conditions describe different clusters. Marfan syndrome tends to show tall stature, long fingers, and aortic root widening rather than marked skin hyperextensibility and joint hypermobility. Scleroderma features skin thickening and tightness, not the easily stretchable skin and lax joints. Osteogenesis imperfecta focuses on bone fragility and fractures, sometimes blue sclera, but not prominent skin hyperextensibility or joint hypermobility.
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