Segura Saint-Gerons Rafael, Ceballos Salobreña Alejandro, Toro Rojas Mariano, Gándara Rey José Manuel
C.S. Peñarroya, Córdoba.
Med Oral Patol Oral Cir Bucal. 2006 Aug 1;11(5):E421-4.
Cowden's disease, or multiple hamartoma syndrome, is an autosomal dominant genodermatosis, characterized by the presence of multiple cutaneous hamartomas, oral fibromas and benign acral keratosis. It affects multiple organs (breast, thyroids, stomach, colon), with the strong possibility of malignant neoplasia developing in these organs. We present a case of this rare syndrome, highlighting the presentation of some clinical characteristics that, in suspected cases, can help to establish an early diagnosis of this disease, this being of great importance given the high frequency of tumors in people with this clinical picture.
考登病,即多发性错构瘤综合征,是一种常染色体显性遗传性皮肤病,其特征为存在多发性皮肤错构瘤、口腔纤维瘤和良性肢端角化病。它会影响多个器官(乳房、甲状腺、胃、结肠),这些器官发生恶性肿瘤的可能性很大。我们报告了一例这种罕见综合征的病例,突出了一些临床特征的表现,在疑似病例中,这些特征有助于对该病进行早期诊断,鉴于具有这种临床表现的人群中肿瘤发生率很高,这一点非常重要。