Human immunodeficiency virus (HIV)-related vascular diseases with skin manifestations are varied. The most common and well-studied disorder in this group, Kaposi’s sarcoma (KS), is discussed in Chapter 12, as are the so-called angiocentric lymphoproliferative disorders. What remains is a broad array of rare disorders, often with bizarre clinical manifestations. As demonstrated below, the breadth of vascular disease results, in part, from the direct effects of HIV infection and, more commonly, from indirect consequences, such as immune suppression.