title: Phakomatosis pigmentovascularis and Lisch nodules. Relationship between Von Recklinghausen and phakomatosis pigmentovascularis?
abstract: In 1947, Ota described a malformative syndrome associating a vascular component (nevus flammeus) with melanocytic or epidermic nevi, which he named phakomatosis pigmentovascularis (PPV). We will discuss the case of a 10-year-old boy presenting giant nevus flammeus, nevus spillus, asymmetry in the development of both lower limbs, characteristic of PPV, together with interventricular communication and Lisch nodules, representative of Von Recklinghausen's disease. We believe that this case can be classified as PPV type IIb, associated with Lisch nodules and rarely described in Caucasian individuals.
authors.affiliation: Department of Dermatology, Hospital Universitario San Cecilio, Granada, Spain.
authors.forename: R
authors.initials: R
authors.lastname: Ruiz Villaverde
authors.forename: A
authors.initials: A
authors.lastname: Viera Ramirez
authors.forename: J
authors.initials: J
authors.lastname: Linares Solano
authors.forename: R
authors.initials: R
authors.lastname: Naranjo Sintes
authors.forename: M T
authors.initials: MT
authors.lastname: Gutierrez Salmerón
mesh_terms: D002648:Child; D006801:Humans; D015811:Iris Neoplasms / Q000175:diagnosis*; D008297:Male; D020752:Neurocutaneous Syndromes / Q000175:diagnosis*; D009456:Neurofibromatosis 1 / Q000175:diagnosis*; D009508:Nevus, Pigmented / Q000175:diagnosis*; D019339:Port-Wine Stain / Q000175:diagnosis*; D012878:Skin Neoplasms / Q000175:diagnosis*