Author: Nat Pernick, M.D., PathologyOutlines.com, Inc.
Revised: 7 July 2009, last major update June 2009
● Rare syndrome of widespread, aberrant and persistent nevus flammeus (vascular nevus, capillary malformation) and extensive pigment abnormalities
● Divided into three main types:
● Phacomatosis cesioflammea (blue spots [caesius = bluish gray] plus nevus flammeus) - also called types IIa/IIb; includes nevus of Ota; most common of 3 types (J Am Acad Dermatol 2008;58:88); often associated with Sturge-Weber and Klippel-Trenaunay syndromes
● Phacomatosis spilorosea (nevus spilus [speckled lentiginous nevus] plus pale-pink telangiectatic nevus) - also called types IIIa/IIIb
● Phacomatosis cesiomarmorata (blue spots plus cutis marmorata telangiectatica congenita) - also called type V
● Subtypes: a - cutaneous involvement only, b - cutaneous and system involvement
● Cutis tricolor coexistent with cutis marmorata telangiectatica congenita (Eur J Dermatol 2008;18:394)
Phacomatosis spilorosea Phacomatosis cesiomarmorata
End of Skin-Melanocytic Tumors > Phacomatosis pigmentovascularis
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