Abstract

LAVIN CASTEJON, I.; MUT OLTRA, J.; TRILLO BALIZON, C.  and  MALDONADO BARRIONUEVO, A.. Lhermitte-Duclos´s disease associated to Cowden´disease: a case report. An. Med. Interna (Madrid) [online]. 2007, vol.24, n.5, pp.239-241. ISSN 0212-7199.

Cowden's disease is a rare genodermatosis that is characterized for multiple cutaneus 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 ussually 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 performanced for intense cephalea, and it was found a bad-defined mass in right cerebelous hemisphere without contrast captation, compatible with cerebelous glangliocytoma.

Keywords : Lhermitte-Duclos´s disease; Displasic gangliocytoma; Cowden's disease; Multiple hamartomatosis; Gastrointestinal poliposis; MRN.

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