A 1-month-old boy was evaluated for a "refractory diaper rash." He had been born at full term after ian uneventful gestation. During the newborn period, he was noted to have multiple erosions and ulcers in the perineal area that were diagnosed as severe diaper rash. Examination of external genitalia revealed a bifid scrotum. His anus was displaced anteriorly and toward the left, and a right-sided cutaneous recess resembling an anus was identified. Purplish discoloration (erythema and telangiectasia) in the perineal area was observed. This lesion gradually increased in size and became a perineal hemangioma (Figure). The patient's constellation of findings led to a diagnosis of mild variant of PELVIS (perineal hemangioma, external genital malformations, lipomyelomeningocele, vesicorenal abnormalities, imperforate anus, and skin tag) syndrome. Magnetic resonance imaging confirmed the presence of a perineal hemangioma and showed no gross anomalies in other visceral structures, including spine and urinary tract. Imperforate anus was surgically corrected at 2 months of age.
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