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Linear porokeratosis associated with Bardet‐Biedl syndrome: A case report
Author(s) -
Tappel Anna C.,
Tiwari Nitin,
Zlotoff Barrett
Publication year - 2019
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.13776
Subject(s) - medicine , porokeratosis , trunk , immunosuppression , dermatology , bardet–biedl syndrome , genetics , phenotype , ecology , biology , gene
This case report presents a 17‐year‐old boy with Bardet‐Biedl syndrome ( BBS ) and a long‐standing hyperpigmented eruption on the left trunk and upper extremity, clinically and histologically consistent with linear porokeratosis ( LP ). BBS patients frequently require solid organ transplant, and subsequent immunosuppression places them at especially high risk for malignant transformation of premalignant skin lesions such as LP . Although BBS affects multiple organ systems, there are only a handful of case reports detailing associated cutaneous involvement, and, to our knowledge, this is the first reported case of linear porokeratosis occurring in patient with BBS .