Abstract：
A 17-year-old male presented for review of a widespread keratinocytic epidermal nevus (KEN) in the setting of a chronic pericardial effusion. Biopsy of the epidermal nevus revealed a KRAS mutation. Pericardiocentesis revealed a chylous effusion and magnetic resonance lymphangiogram demonstrated an underlying lymphatic malformation. There are rare case reports of KEN with an associated KRAS mutation. This case highlights the importance of being alert to epidermal nevus syndrome, particularly in patients with a widespread nevus and seemingly unrelated pathology.
摘要：
一名17岁的男性在慢性心包积液的情况下接受了广泛的角质形成表皮痣（KEN）的审查。表皮痣的活检显示KRAS突变。心包穿刺术显示乳糜积液，磁共振淋巴管图显示潜在的淋巴畸形。有罕见的KRAS突变的KEN病例报告。这个案例突出了警惕表皮痣综合征的重要性，特别是在患有广泛痣和看似无关的病理的患者中。
