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Skin-Melanocytic tumors
Phacomatosis pigmentokeratotica
Author: Nat Pernick, M.D., PathologyOutlines.com, Inc.
Revised: 7 July 2009, last major update June 2009
Definition
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● Rare syndrome defined by organoid nevus (occasionally with sebaceous differentiation), speckled lentiginous nevus and other extracutaneous anomalies
● May be caused by twin-spot phenomenon (2 different mutant patches involving 2 adjacent or corresponding areas of the body, Arch Dermatol 1998;134:333, Am J Med Genet 1999;85:355)
Case reports
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● Malignant degeneration of both nevus components (Pediatr Dermatol 2005;22:44)
Clinical images
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Speckled lentiginous nevus showing a checkerboard distribution and
hemiatrophy with postural deviation
Linear arrangement of organoid Systematized epidermal
nevus on neck and upper back nevus of left side of body
Speckled lentiginous nevus Epidermal nevus and
in lumbar area pigmented lesions
Epidermal nevus right side of trunk Various images
and leg plus epidermal nevus and
melanocytic nevus right hand
End of Skin-Melanocytic Tumors > Phacomatosis pigmentokeratotica
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