Gass J D, Gieser R G, Wilkinson C P, Beahm D E, Pautler S E
Department of Ophthalmology, Bascom Palmer Eye Institute, University of Miami, Fla.
Arch Ophthalmol. 1990 Apr;108(4):527-33. doi: 10.1001/archopht.1990.01070060075053.
The development of multiple, round or oval, subtle, red patches at the level of the pigment epithelium in the posterior ocular fundus and their striking early hyperfluorescence angiographically are characteristic features of the bilateral diffuse uveal melanocytic proliferation syndrome. They may be accompanied by severe visual loss and may antedate the appearance of multiple melanocytic tumors, retinal detachment, and cataract in these patients with occult systemic carcinomas. These hyperfluorescent patches are caused by focal damage to the pigment epithelium overlying an intact choriocapillaris and diffuse benign nonpigmented uveal melanocytic infiltration of the outer choroid. We suggest that outer retinal damage may not be primarily caused by melanocytic proliferation, but rather by toxic and immune factors generated by interaction between a systemic carcinoma and congenital melanocytosis of the uveal tract. We report the longest survivor of this disorder to date (102 months).
双侧弥漫性葡萄膜黑素细胞增生综合征的特征性表现为，在眼后极部色素上皮层出现多个圆形或椭圆形、细微的红色斑片，以及血管造影早期显著的高荧光。在这些隐匿性系统性癌患者中，它们可能伴有严重视力丧失，且可能早于多发性黑素细胞瘤、视网膜脱离和白内障的出现。这些高荧光斑片是由覆盖完整脉络膜毛细血管的色素上皮局灶性损伤以及脉络膜外层弥漫性良性无色素葡萄膜黑素细胞浸润所致。我们认为，视网膜外层损伤可能并非主要由黑素细胞增生引起，而是由系统性癌与葡萄膜先天性黑素细胞增多症相互作用产生的毒性和免疫因素所致。我们报告了迄今为止该疾病最长存活者（102个月）。
