A 75-year-old man presented with a 4-month history of edema, erythema and progressive skin thickening affecting the limbs. No triggers were identified. His past medical history was irrelevant. The physical examination revealed induration in the upper and lower limbs, sparing the hands, feet, face and trunk. Linear depressions along the course of the veins, exacerbated by limb elevation, were present on the forearms, and were consistent with the groove sign (Fig. 1).

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