Acute abdomen in a patient with undiagnosed complete androgen insensitivity

We describe a patient with complete androgen insensitivity diagnosed incide ntally after hospitalization for acute abdomen. Androgen insensitivity was assumed because axillary and pubic hair were absent. Increasing abdominal p ain was caused by torsion of the suspensory ligament of the left testis. im mediate surgery was required and both testes were removed. Plasma testoster one levels dropped from 6.6 to 0.34 ng/ml. Karyotyping revealed a normal ma le pattern 46 XY. Genomic sequencing of exon 3 of the androgen receptor det ected a TGT to TAT transition at the DNA-binding domain causing an amino ac id change from cysteine to tyrosine at position 611. This mutation caused a complete defect of the androgen receptor. Oestrogen replacement therapy wa s begun.