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Phacomatosis pigmentokeratotica: Exploring extracutaneous comorbidities and topical therapy.
Pediatric Dermatology 2024 April 16
Phacomatosis pigmentokeratotica (PPK) is a RASopathy characterized by the presence of a sebaceous nevus and a papular speckled lentiginous nevus. This case report highlights the associated extracutaneous comorbidities, including life-threatening arrhythmia, and introduces topical rapamycin as a potential therapeutic avenue for sebaceous nevus in PPK patients.
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