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Year : 2019  |  Volume : 20  |  Issue : 3  |  Page : 267-270

A rare phenomenon of twin spotting: Phacomatosis pigmentokeratotica

Department of Dermatology, Venereology and Leprosy, Government Medical College, Nagpur, Maharashtra, India

Correspondence Address:
Dr. Vrutika Hasmukh Shah
B.1103, Apollo Pride Enclave Complex, Next to Thane Bharat Sahakari Bank, Baji Prabhu Deshpande Marg, Vishnunagar, Thane West, Thane - 400 602, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijpd.IJPD_98_18

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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.

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