A rare phenomenon of twin spotting: Phacomatosis pigmentokeratotica

Phacomatosis pigmentokeratotica is a rare form of cutaneous mosaicism characterized by epidermal nevus usually nevus sebaceous, papular speckled lentiginous nevus, and extracutaneous anomalies. This type of twin-spot phenomenon (didymosis) is due to postzygotic crossing-over resulting in two homozyg...

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Bibliographic Details
Main Authors: Vaishali Wankhade, Vrutika Hasmukh Shah, Rajesh P Singh, Jayesh Mukhi
Format: Article
Language:English
Published: Wolters Kluwer Medknow Publications 2019-01-01
Series:Indian Journal of Paediatric Dermatology
Subjects:
Online Access:http://www.ijpd.in/article.asp?issn=2319-7250;year=2019;volume=20;issue=3;spage=267;epage=270;aulast=Wankhade
Description
Summary:Phacomatosis pigmentokeratotica is a rare form of cutaneous mosaicism characterized by epidermal nevus usually nevus sebaceous, papular speckled lentiginous nevus, and extracutaneous anomalies. This type of twin-spot phenomenon (didymosis) is due to postzygotic crossing-over resulting in two homozygous daughter cells, representing the stem cells of the two distinct types of nevi and leading to additional extracutaneous defects. We report a case of this rare syndrome in a 4-year-old male child associated with skeletal anomalies.
ISSN:2319-7250