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Case Reports in Dermatological Medicine
Volume 2014, Article ID 437085, 3 pages
Case Report

Vascular Malformation and Common Keratinocytic Nevus of the Soft Type: Phacomatosis Pigmentovascularis Revisited

Department of Dermatology and Venereology, Hospital de Curry Cabral, Centro Hospitalar de Lisboa Central, Rua da Beneficência, No. 8, 1069-166 Lisboa, Portugal

Received 24 June 2014; Revised 5 November 2014; Accepted 10 November 2014; Published 23 November 2014

Academic Editor: Kowichi Jimbow

Copyright © 2014 André Laureano et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.


Phacomatosis pigmentovascularis is a rare syndrome characterized by the coexistence of a pigmented nevus and a cutaneous vascular malformation. We report a 5-year-old boy with all the typical findings of phacomatosis pigmentovascularis type Ia. Although its existence according to the traditional classification has been questioned, this case represents a very rare association of a capillary vascular malformation and a common keratinocytic nevus of the soft type.