PDF(Pubmed)
Abstract：
A 56-year-old Brazilian woman sought dental care, presenting with multiple asymptomatic papillomatous lesions with a coalescent pattern and intermingled cobblestone-like clefts along the alveolar ridge and marginal and attached gingivae. Multiple whitish papules were also observed on the face, neck, and limbs. Incisional biopsies of these lesions were performed. Microscopically, the skin lesion revealed epithelial clear cells and intraepithelial keratinization with areas of orthokeratosis, while the gingival lesions showed a parakeratinized stratified squamous epithelium with collagenous connective tissue. These features were consistent with those of a trichilemmoma and fibroepithelial hyperplasia, respectively. This article illustrates a case of Cowden syndrome (CS), a rare multisystem genetic condition in which both cutaneous and mucosal tissues were affected. Fewer than 40 cases of CS with oral involvement affecting middle-aged adults have been documented hitherto.
摘要：
一名56岁的巴西妇女寻求牙科护理，表现为多个无症状的乳头状瘤性病变，呈合并模式，并沿着牙槽脊和边缘和附着的牙龈混合在一起。脸上还观察到多个发白的丘疹，脖子,和四肢。对这些病变进行了切开活检。微观上,皮肤病变显示上皮透明细胞和上皮内角化，并伴有角化区，而牙龈病变表现为角化复层鳞状上皮和胶原结缔组织。这些特征与毛癣瘤和纤维上皮增生的特征一致，分别。本文说明了一例Cowden综合征(CS)，一种罕见的多系统遗传状况，皮肤和粘膜组织均受到影响。迄今为止，已有文献记载的影响中年人的口腔受累CS病例不到40例。
