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Phacomatosis pigmentokeratotica: Exploring extracutaneous comorbidities and topical therapy.
Camiña-Conforto, Gemma; Ivars, Marta; Sarquella-Brugada, Georgia; Valera-Dávila, Carlos; Salvador, Héctor; Rovira, Carlota; Baselga, Eulalia.
Affiliation
  • Camiña-Conforto G; Department of Dermatology, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain.
  • Ivars M; Department of Dermatology, Inherited Cardiac Diseases and Sudden Death Unit, Barcelona, Spain.
  • Sarquella-Brugada G; Department of Arrhythmia, Inherited Cardiac Diseases and Sudden Death Unit, Barcelona, Spain.
  • Valera-Dávila C; Department of Neurology, Hospital Sant Joan de Déu de Barcelona, Barcelona, Spain.
  • Salvador H; Department of Pediatric Oncology, Hospital Sant Joan de Déu de Barcelona, Barcelona, Spain.
  • Rovira C; Department of Pathology, Hospital Sant Joan de Déu de Barcelona, Barcelona, Spain.
  • Baselga E; Department of Dermatology, Inherited Cardiac Diseases and Sudden Death Unit, Barcelona, Spain.
Pediatr Dermatol ; 2024 Apr 15.
Article in En | MEDLINE | ID: mdl-38621679
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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Full text: 1 Collection: 01-internacional Database: MEDLINE Language: En Journal: Pediatr Dermatol Year: 2024 Type: Article Affiliation country: Spain
Fulltext
Print
XML
PubMed Links
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Full text: 1 Collection: 01-internacional Database: MEDLINE Language: En Journal: Pediatr Dermatol Year: 2024 Type: Article Affiliation country: Spain