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.


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.


CONCLUSIONS
It is suggested that this association is not a coincidence, but is part of a neurocutaneous syndrome which has not been described previously.
Valdivieso
Rommel
R
Centro de Alergia y Dermatología, Quito, Ecuador. rommelva@andinanet.net
D000236:Adenoma / Q000150:complications*; D000328:Adult; D019080:Cafe-au-Lait Spots / Q000150:complications*; D003638:Deafness / Q000150:complications* / Q000151:congenital; D005260:Female; D018219:Histiocytoma, Benign Fibrous / Q000150:complications; D006801:Humans; D008159:Lumbar Vertebrae / Q000002:abnormalities; D020752:Neurocutaneous Syndromes / Q000175:diagnosis*; D009508:Nevus, Pigmented / Q000150:complications; D010911:Pituitary Neoplasms / Q000150:complications*; D012878:Skin Neoplasms / Q000150:complications