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
Published inIndian journal of paediatric dermatology Vol. 20; no. 3; pp. 267 - 270
Main Authors Wankhade, Vaishali, Shah, Vrutika, Singh, Rajesh, Mukhi, Jayesh
Format Journal Article
LanguageEnglish
Published Wolters Kluwer India Pvt. Ltd 01.07.2019
Medknow Publications and Media Pvt. Ltd
Wolters Kluwer Medknow Publications
Subjects
Analysis
Care and treatment
Child health
Cutaneous mosaicism
Medical research
Nevus
nevus sebaceous
Rare diseases
skeletal
speckled lentiginous nevus
Stem cells
twin-spot phenomenon
speckled lentiginous nevus
twin-spot phenomenon
Cutaneous mosaicism
skeletal
nevus sebaceous
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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.
ISSN:2319-7250
2319-7269
DOI:10.4103/ijpd.IJPD_98_18