Torrelo Antonio, Boente María del C, Nieto Olga, Asial Raúl, Colmenero Isabel, Winik Beatriz, Zambrano Antonio, Happle Rudolf
Department of Dermatology, Hospital del Nino Jesús, Madrid, Spain.
Pediatr Dermatol. 2005 May-Jun;22(3):206-9. doi: 10.1111/j.1525-1470.2005.22305.x.
Nevus psiloliparus is a distinct type of mesodermal nevus of the scalp characterized by absence or paucity of hair, and presence of an excessive amount of fatty tissue. It is considered a hallmark of encephalocraniocutaneous lipomatosis, a rare disorder comprising a variety of cutaneous, ophthalmologic, and neurologic defects. We report two infants with encephalocraniocutaneous lipomatosis with nevus psiloliparus on the scalp in close association with aplasia cutis congenita. This unusual association may be considered a further example of didymosis, for which we propose the term, didymosis aplasticopsilolipara.
秃发性脂性痣是头皮中一种独特的中胚层痣，其特征是毛发缺失或稀少，以及存在过量的脂肪组织。它被认为是脑颅皮肤脂肪瘤病的一个标志，这是一种罕见的疾病，包括各种皮肤、眼科和神经缺陷。我们报告了两名患有脑颅皮肤脂肪瘤病的婴儿，其头皮上有秃发性脂性痣，与先天性皮肤发育不全密切相关。这种不寻常的关联可能被认为是联体畸形的又一个例子，为此我们提出“先天性皮肤发育不全性秃发性脂性联体畸形”这一术语。
