Publication: Phacomatosis pigmentokeratotica: A further case without extracutaneous anomalies and review of the condition
Issued Date
2011-11-01
Resource Type
ISSN
15251470
07368046
07368046
Other identifier(s)
2-s2.0-81255201145
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Mahidol University
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SCOPUS
Bibliographic Citation
Pediatric Dermatology. Vol.28, No.6 (2011), 715-719
Suggested Citation
Rattanavalai Chantorn, Tor Shwayder Phacomatosis pigmentokeratotica: A further case without extracutaneous anomalies and review of the condition. Pediatric Dermatology. Vol.28, No.6 (2011), 715-719. doi:10.1111/j.1525-1470.2011.01655.x Retrieved from: https://repository.li.mahidol.ac.th/handle/123456789/12247
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Title
Phacomatosis pigmentokeratotica: A further case without extracutaneous anomalies and review of the condition
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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. © 2011 Wiley Periodicals, Inc.