Author: Nat Pernick, M.D., PathologyOutlines.com, Inc.
Revised: 7 July 2009, last major update June 2009
● Rare syndrome defined by organoid nevus (occasionally with sebaceous differentiation), speckled lentiginous nevus and other extracutaneous anomalies
● Malignant degeneration of both nevus components (Pediatr Dermatol 2005;22:44)
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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