Ipsilateral café-au-lait macules, deafness, and pituitary microadenoma in a 35-year-old woman.

BACKGROUND

The association between ipsilateral café-au-lait macules, deafness, and pituitary adenoma has not been described previously.

CASE REPORT AND RESULTS

A 35-year-old woman affected since birth with two café-au-lait macules, one measuring 15 x 20 cm, with an irregular shape, covering almost all of the left buttock, and the other measuring 6 x 13 cm on the front side of the left thigh, is described. For as long as the patient can remember, she has been totally deaf in the left ear, diagnosed as 100% congenital sensorineural deafness. In addition, a left-side pituitary microadenoma was detected at the age of 31 years. She was also found to have a transitional lumbosacral vertebra, and multiple dermatofibromas and nevomelanocytic nevi.

CONCLUSION

It is suggested that this association is not a coincidence, but is part of a neurocutaneous syndrome which has not been described previously.