Phacomatosis Pigmentokeratotica: A Further Case without Extracutaneous Anomalies and Review of the Condition
Article first published online: 15 NOV 2011
© 2011 Wiley Periodicals, Inc.
Volume 28, Issue 6, pages 715–719, November/December 2011
How to Cite
Chantorn, R. and Shwayder, T. (2011), Phacomatosis Pigmentokeratotica: A Further Case without Extracutaneous Anomalies and Review of the Condition. Pediatric Dermatology, 28: 715–719. doi: 10.1111/j.1525-1470.2011.01655.x
- Issue published online: 15 NOV 2011
- Article first published online: 15 NOV 2011
Abstract: Epidermal nevus syndrome is the term for the association of an epidermal nevus and extracutaneous anomalies, including neurologic, ophthalmic, and skeletal defects. Epidermal nevus syndromes include different disorders that share the feature of mosaicism. Phacomatosis pigmentokeratotica (PPK) is a distinctive new epidermal nevus syndrome first described in 1996 characterized by the presence of multiple organoid nevi with sebaceous differentiation, a speckled lentiginous nevus, and skeletal and neurologic abnormalities. Only a handful of cases of PPK without extracutaneous manifestations have been reported. We report here an individual with PPK with only cutaneous signs and confirm this distinctive syndrome has two subtypes according to the presence or absence of extracutaneous involvement.