Classification of phakomatosis pigmentovascularis

Type I	Capillary malformation + epidermal nevus
Type II	Capillary malformation + dermal melanocytosis +/– nevus anemicus
Type III	Capillary malformation + nevus spilus +/– nevus anemicus
Type IV	Capillary malformation + dermal melanocytosis + nevus spilus +/– nevus anemicus

Original table modified for this publication. Reproduced from: Paller AS, Mancini AJ. Vascular disorders of childhood. In: Hurwitz Clinical Pediatric Dermatology, 3rd ed, Elsevier, 2006. Table used with the permission of Elsevier Inc. All rights reserved.

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Classification of phakomatosis pigmentovascularis