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Anais Brasileiros de Dermatologia

On-line version ISSN 1806-4841

Abstract

VETTORATO, Gerson; SOUZA, Paulo Ricardo Martins; BOZKO, Márcia Paczco  and  LAMB, Flávia Maria. Cowden's disease or mutiple hamartoma syndrome. An. Bras. Dermatol. [online]. 2003, vol.78, n.2, pp. 209-213. ISSN 1806-4841.  http://dx.doi.org/10.1590/S0365-05962003000200008.

A case of Cowden's disease or Multiple Hamartoma Syndrome is reported. The disorder is inherited as an autosomal dominant trait, the classic dermatological features of which are multiple facial trichilemmomas (hamartomas of the follicular infundibula), oral fibroma and benign acral keratosis. Multiple organs are affected and it is associated with mama, thyroid and colon malignant neoplasms. We present a young woman with skin-colored flat-topped papules in the central facial area, papular gingival and palatal lesions, fissured tongue, palmoplantar keratoses and prior history of subtotal thyroidectomy.

Keywords : Multiple hamartoma syndrome; Mouth diseases.

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