Epithelioid angiosarcoma is a rare aggressive vascular malignancy with a high recurrence and metastasis rate. Most commonly arising in the deep soft tissues while cutaneous localizations are extremely rare. We report here a case of epithelioid angiosarcoma in a young man with a specific context of lower limbs chronic lymphedema, showing clinical dermoscopic and pathology findings. Epithelioid angiosarcome is a highly malignant tumor. An early clinical and dermoscopic examination can improve the prognosis and avoid mutilating complications.

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