[Lhermitte-Duclos's disease associated to Cowden s disease: a case report]. / Enfermedad de Lhermitte-Duclos asociada a enfermedad de Cowden: a propósito de un caso.

ABSTRACT

Cowden's disease is a rare genodermatosis that is characterized for multiple cutaneous and visceral hamartoma . Lhermitte-Duclos's disease is a cerebelous lesion that consists in the displasic enlargement of the cerebelous circumvolution. It's incluyed in phacomatosis and usually presents associated to Cowden's disease, tuberous sclerosis and overlap syndromes.A 56 years old man, diagnosed in Dermatology with Cowden's disease ten years ago. In the extension study, he had hamartoma intestinal polip, esophagic glucogenic acanthosis and two solid thyroid nodules. The craneal TC didn't show significant alteration. Ten years after diagnosis a cerebral magnetis resonance was performed for intense cephalea, and it was found a bad-defined mass in right cerebelous hemisphere without contrast captation, compatible with cerebelous glangliocytoma.