Gumaste Priyanka, Ortiz Arisa E, Patel Amit, Baron Jonathan, Harris Ronald, Barr Ronald
*Rutgers-Robert Wood Johnson Medical School, New Brunswick, NJ; †Department of Dermatology, Dermatology Cosmetic and Laser Center, Massachusetts General Hospital, Harvard Medical School, Boston, MA; ‡Department of Medicine-Division of Dermatology, UC San Diego, San Diego, CA; §Department of Dermatology, UC Irvine, Irvine, CA; ¶RBGB Dermatology, Santa Ana, CA; and ‖Laguna Pathology Medical Group, Laguna Beach, CA.
Am J Dermatopathol. 2015 Mar;37(3):e37-40. doi: 10.1097/DAD.0000000000000085.
Basaloid follicular hamartoma (BFH) is a rare, generally benign lesion of importance because of its clinical and histopathological similarity to infundibulocystic basal cell carcinoma. Autosomal dominant generalized BFH syndrome is 1 of the 5 clinical forms of BFH that has been described in the literature. We report a case of BFH syndrome in a 47-year-old Hispanic female who presented with an increasing number of small 1- to 2-mm tan to brown smooth facial papules, few palmar pits, and cobblestoning of the tongue. Her mother had similar lesions on her face. A biopsy of one of the patient's facial lesions confirmed the diagnosis of BFH. Of note, this patient later presented with rapid growth of one of her facial lesions, and a subsequent biopsy confirmed the development of a basal cell carcinoma arising within one of her BFH lesions. Although BFH is classically stable for years and does not require immediate surgical removal, our case highlights the importance of continual monitoring of these patients, given the potential for malignant transformation of these lesions.
基底样滤泡性错构瘤(BFH)是一种罕见的、通常为良性的病变,因其临床和组织病理学表现与漏斗状囊性腺样基底细胞癌相似而受到重视。常染色体显性遗传性全身性BFH综合征是文献中描述的BFH的5种临床类型之一。我们报告了一例47岁西班牙裔女性的BFH综合征病例,该患者面部出现越来越多1至2毫米大小的黄褐色至棕色光滑丘疹,少量掌跖凹陷,以及舌部鹅卵石样改变。她的母亲面部有类似病变。对患者面部一处病变进行活检确诊为BFH。值得注意的是,该患者后来面部一处病变迅速生长,随后活检证实其BFH病变之一发生了基底细胞癌。虽然经典的BFH多年来通常稳定,不需要立即手术切除,但我们的病例强调了持续监测这些患者的重要性,因为这些病变有恶变的可能。