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Phacomatosis pigmentokeratotica: Exploring extracutaneous comorbidities and topical therapy
Gemma Camiña-Conforto1, Marta Ivars2, Georgia Sarquella-Brugada3
1Department of Dermatology, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain.
Abstract:
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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