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Nailfold scleroderma-like capillary abnormalities in Werner syndrome (adult progeria)

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A 49-year-old male was referred to our rheumatology division for suspicion of a scleroderma spectrum disorder. He spontaneously suffered from large, deep ulcers on both legs, around the left Achilles… Click to show full abstract

A 49-year-old male was referred to our rheumatology division for suspicion of a scleroderma spectrum disorder. He spontaneously suffered from large, deep ulcers on both legs, around the left Achilles tendon, lateral malleolus and elbows (Panels A1 and A2). Lower extremity pulse examination and vascular Doppler ultrasound were normal. A complete examination showed scleroderma-like skin involvement of the abdomen and lower and upper limbs, except for the hands. Furthermore, flexion contractures of the wrists, ankles and feet, and lower limb paresthesia were detected. He also reported xerostomia and xerophthalmia. His medical history was notable for long-standing progressive dysphonia that had evolved into a high-pitched hoarse voice. Moreover, he had bilateral cataract surgery at 18 years of age, osteopenia, premature hair loss and graying, and short stature. Raynaud’s phenomenon and telangiectasias were not present. Further investigations, including autoantibodies, were all negative except for mild hypercholesterolemia, and no organ involvement was identified. Nailfold capillary Nailfold scleroderma-like capillary abnormalities in Werner syndrome (adult progeria)

Keywords: abnormalities werner; nailfold scleroderma; capillary abnormalities; werner syndrome; scleroderma like; like capillary

Journal Title: Vascular Medicine
Year Published: 2017

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