de Luna M L, Barquin M A, Casas J G, Sidelsky S
Department of Pediatric Dermatology, Buenos Aires University, Hospital General de Agudos Jose Maria Ramos Mejía, Argentina.
Pediatr Dermatol. 1995 Jun;12(2):159-63. doi: 10.1111/j.1525-1470.1995.tb00145.x.
We report a 1-year-old boy with an extensive cutaneous vascular malformation, oculocutaneous pigmentation, and severe neurologic abnormalities from birth, as well as a selective IgA deficiency. Ultrastructural study demonstrated prominent endothelial cells in the luminal of the blood vessels. The diagnosis of phacomatosis pigmentovascularis type IIb seemed appropriate for this patient.
我们报告一名1岁男童,自出生起即患有广泛的皮肤血管畸形、眼皮肤色素沉着和严重的神经异常,以及选择性IgA缺乏症。超微结构研究显示血管腔内有突出的内皮细胞。IIb型色素血管性错构瘤病的诊断似乎适用于该患者。