title: Palmoplantar Keratoderma in Costello Syndrome Responsive to Acitretin.
abstract: Costello syndrome (CS) is a multisystem congenital disorder characterized by coarse facial features, cardiac defects, intellectual disability, and predisposition to malignancies. Dermatologic findings can include cutaneous papillomas, skin redundancy, acanthosis nigricans, and keratosis pilaris. Palmoplantar keratoderma (PPK) is present in approximately 76% of patients with CS, with disabling functional consequences in severe cases. We report a case of CS with severe PPK that improved dramatically with systemic administration of acitretin 0.3 mg/kg/day.
authors.affiliation: Department of Dermatology, School of Medicine, Yale University, New Haven, Connecticut.
authors.forename: Nareh V
authors.initials: NV
authors.lastname: Marukian
authors.affiliation: Department of Dermatology, School of Medicine, Yale University, New Haven, Connecticut.
authors.forename: Jonathan L
authors.initials: JL
authors.lastname: Levinsohn
authors.affiliation: Department of Dermatology, School of Medicine, Yale University, New Haven, Connecticut.|Department of Pediatrics, School of Medicine, Yale University, New Haven, Connecticut.
authors.forename: Brittany G
authors.initials: BG
authors.lastname: Craiglow
authors.affiliation: Department of Dermatology, School of Medicine, Yale University, New Haven, Connecticut.
authors.forename: Leonard M
authors.initials: LM
authors.lastname: Milstone
authors.affiliation: Department of Dermatology, School of Medicine, Yale University, New Haven, Connecticut.|Department of Genetics, School of Medicine, Yale University, New Haven, Connecticut.|Department of Pathology, School of Medicine, Yale University, New Haven, Connecticut.
authors.forename: Keith A
authors.initials: KA
authors.lastname: Choate
mesh_terms: D017255:Acitretin / Q000627:therapeutic use*; D056685:Costello Syndrome / Q000150:complications* / Q000473:pathology*; D005260:Female; D006801:Humans; D007645:Keratoderma, Palmoplantar / Q000188:drug therapy* / Q000209:etiology* / Q000473:pathology; D007641:Keratolytic Agents / Q000627:therapeutic use*; D055815:Young Adult
chemical_list: D007641:Keratolytic Agents; D017255:Acitretin