Fabry病,又称为弥漫性躯体血管角皮瘤(angiokeratoma corporis diffusum,ACD)是一罕见的伴性隐性遗传性神经皮肤综合征,国内仅有5例报告,且均为皮肤科临床所见。本文报告以肢端疼痛为主要表现的两家系共5例。先证者1 男,21岁,9岁起阵发性四肢末端疼痛 Fabry’s disease, also known as angiokeratoma corporis diffusum (ACD), is a rare complication of recessive hereditary neurodermatosis. There are only 5 cases reported in China, and all of them are found in clinical dermatology . This article reports acromegaly as the main performance of the two families in 5 cases. Probate 1 male, 21 years old, 9 years old paroxysmal extremity pain