Orofacial Manifestations in a Middle-Aged Woman with Cowden Syndrome: A Case Image

被引：3

作者：

de Arruda, Jose Alcides Almeida ^{[1
]}

Freire, Clara Herrera ^{[2
]}

Leite, Taisa Domingues Boehmer ^{[2
]}

Noce, Cesar Werneck ^{[3
]}

Cavalcante, Israel Leal ^{[2
,4
]}

Vogel, Jessica de Oliveira ^{[2
]}

Mesquita, Ricardo Alves ^{[1
]}

Tenorio, Jefferson R. ^{[2
]}

de Andrade, Bruno Augusto Benevenuto ^{[2
]}

机构：

[1] Univ Fed Minas Gerais, Sch Dent, Dept Oral Surg Pathol & Clin Dent, Av Pres Antonio Carlos,6627,Room 3202 D Pampulha, Belo Horizonte, MG, Brazil

[2] Univ Fed Rio De Janeiro, Sch Dent, Dept Oral Diag & Pathol, Rio De Janeiro, Brazil

[3] Hosp Forca Aerea Galeao, Oral Med Serv, Rio De Janeiro, Brazil

[4] Univ Fortaleza, Dept Dent, Fortaleza, Brazil

来源：

HEAD & NECK PATHOLOGY | 2023年 / 17卷 / 04期

关键词：

Cowden syndrome; Dental care; Multiple hamartoma syndromes; Oral manifestations; PTEN hamartoma tumor syndrome; MUTATIONS;

D O I：

10.1007/s12105-023-01586-2

中图分类号：

R36 [病理学];

学科分类号：

100104 ;

摘要：

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.

引用

页码：1071 / 1074

页数：4

共 6 条

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