Premium
Palmoplantar Keratoderma in Costello Syndrome Responsive to Acitretin
Author(s) -
Marukian Nareh V.,
Levinsohn Jonathan L.,
Craiglow Brittany G.,
Milstone Leonard M.,
Choate Keith A.
Publication year - 2016
Publication title -
pediatric dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.542
H-Index - 73
eISSN - 1525-1470
pISSN - 0736-8046
DOI - 10.1111/pde.13057
Subject(s) - acitretin , palmoplantar keratoderma , medicine , dermatology , acanthosis nigricans , keratoderma , costello syndrome , ichthyosis , dyskeratosis , hyperkeratosis , pityriasis rubra pilaris , pathology , psoriasis , obesity , insulin resistance , colorectal cancer , cancer , kras
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.