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Bannayan‐Riley‐Ruvalcaba syndrome with gingival hyperpigmentation and facial papules

One of the distinctive cutaneous manifestations of Bannayan‐Riley‐Ruvalcaba syndrome (BRRS), a PTEN hamartoma tumor syndrome, is penile pigmented macules. We present a 13‐year‐old boy with gingival hyperpigmentation along with facial and ear angiofibromas in the context of a BRRS‐concordant phenotype and PTEN hamartoma tumor syndrome genotype. To our knowledge, these two findings have not been previously reported with BRRS and may expand the known phenotype of this disorder.