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Phakomatosis pigmentovascularis and Lisch nodules. Relationship between Von Recklinghausen and phakomatosis pigmentovascularis?
Author(s) -
Ruiz Villaverde R,
Viera Ramirez A,
Linares Solano J,
Naranjo Sintes R,
Gutierrez Salmerón MT
Publication year - 2003
Publication title -
journal of the european academy of dermatology and venereology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.655
H-Index - 107
eISSN - 1468-3083
pISSN - 0926-9959
DOI - 10.1046/j.1468-3083.2003.00632.x
Subject(s) - medicine , nevus of ota , dermatology , nevus , pathology , melanoma , cancer research
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.