Papillary transitional cell bladder carcinoma and systematized epidermal nevus syndrome

Epidermal nevus syndrome (ENS), also known as Solomon syndrome, is a rare neurocutaneous disorder defined by mosaicism. Epidermal nevus syndrome may be associated with a variety of systemic findings, several of which have been described in the literature, including but not limited to central nervous system abnormalities and internal malignancies. There is a paucity of reports of patients with both epidermal nevi and papillary transitional cell bladder carcinoma in the literature. We present a rare case of systematized epidermal nevi associated with development of papillary transitional cell bladder carcinoma in a young woman. Patients diagnosed with generalized epidermal nevi should have workup for possible associated central nervous system, musculoskeletal, genitourinary abnormalities, and internal malignancies. The association of epidermal nevi and transitional cell bladder carcinoma may be linked to a mutation in the fibroblast growth factor receptor 3 gene, FGFR₃, but a clear link has yet to be substantiated and additional molecular studies are needed.