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Phacomatosis Pigmentovascularis Revisited and Reclassified

Rudolf Happle, MD
Arch Dermatol. 2005;141(3):385-388. doi:10.1001/archpedi.161.4.356.
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Objective  To provide a new comprehensible and practicable classification by use of descriptive terms to distinguish the various types of phacomatosis pigmentovascularis (PPV), which has previously been classified by numbers and letters that are difficult to memorize.

Study Selection  Published case reports on PPV were reassessed.

Data Extraction and Data Synthesis  A critical review revealed that only 3 well-established types of PPV so far exist. To eliminate the cumbersome traditional classification by numbering and lettering, the following new terms are proposed: phacomatosis cesioflammea (blue spots [caesius = bluish gray] and nevus flammeus); phacomatosis spilorosea (nevus spilus coexisting with a pale-pink telangiectatic nevus), and phacomatosis cesiomarmorata (blue spots and cutis marmorata telangiectatica congenita). Phacomatosis cesioflammea is identical with the traditional types IIa and IIb; phacomatosis spilorosea corresponds to types IIIa and IIIb; and phacomatosis cesiomarmorata is a descriptive term for type V. A categorical distinction of cases with and without extracutaneous anomalies seems inappropriate. The traditional type I does not exist, and the extremely rare traditional type IV is now included in the group of unclassifiable forms.

Conclusion  The proposed new classification of PPV by using 3 descriptive terms may be easier to memorize compared with the time-honored grouping of in part not even existing subtypes by numbers and letters.

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Figure 1.

Phacomatosis cesioflammea: blue spots coexisting with port-wine stains (courtesy of Ramón Ruiz-Maldonado, MD National Institute of Pediatrics, Mexico City, Mexico).

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Figure 2.

Phacomatosis spilorosea: nevus spilus (speckled lentiginous nevus) associated with a pale-pink telangiectatic nevus (reprinted with permission from Hautarzt.2 Copyright 1989, Springer-Verlag GmbH & Co KG).

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Figure 3.

Phacomatosis cesiomarmorata. A, Frontal aspect, showing multiple blue spots and flaglike areas of reticulated, marblelike erythema. B, Dorsal view, showing bluish macules intermingled with areas of cutis marmorata (reprinted with permission from Br J Dermatol.5 Copyright 2003).

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