Basaloid follicular hamartoma with eruptive milia and hypohidrosis: is there a pathogenic relationship ?

We report a sporadic case of eruptive milia with histopathological features of basaloid follicular hamartoma which developed in an 8 year-old Japanese girl. Multiple milia and comedo-like eruptions were present at birth and gradually increased in number and spread over the extremities. Histopathologically, keratotic cysts with trichilemmal keratinization and features of basaloid follicular hamartoma were observed without any histological findings of basal cell epithelioma or trichoepithelioma. Reduced sweating was observed after iontophoretically applied acetylcholine on the forearm. Nevus of Ota and thyroid goiter were complications.