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 (Author), Vrutika Hasmukh Shah (Author), Rajesh P Singh (Author), Jayesh Mukhi (Author)
Format: Book
Published: Wolters Kluwer Medknow Publications, 2019-01-01T00:00:00Z.
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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.
Item Description:2319-7250
10.4103/ijpd.IJPD_98_18