نوع مقاله : Letter to Editor
نویسندگان
1 دانشیار، مرکز تحقیقات پوست و سلول‌های بنیادی، دانشگاه علوم پزشکی تهران، تهران و گروه پوست، دانشکدهی پزشکی، دانشگاه علوم پزشکی اصفهان، ایران
2 دستیار تخصصی، مرکز تحقیقات بیماری های پوستی و سالک، گروه پوست، دانشکده ی پزشکی و کمیته ی تحقیقات دانشجویی، دانشگاه علوم پزشکی اصفهان، اصفهان، ایران
3 استادیار، مرکز تحقیقات بیماری‌های پوستی و سالک و گروه پوست، دانشکده ی پزشکی، دانشگاه علوم پزشکی اصفهان، اصفهان، ایران
چکیده
کلیدواژه‌ها
عنوان مقاله [English]
نویسندگان [English]
Nevoid basal cell carcinoma syndrome (NBCCS), also known as Gorlin syndrome, is a hereditary condition characterized by a wide range of developmental abnormalities and a predisposition to neoplasms. The estimated prevalence varies from 1/57,000 to 1/256,000, with a male-to-female ratio of 1:1. Main clinical manifestations include multiple basal cell carcinomas (BCCs), odontogenic keratocysts of the jaw, hyperkeratosis of palms and soles, skeletal abnormalities, intracranial ectopic calcifications, and facial dysmorphism (macrocephaly, cleft lip/palate and severe eye anomalies). This paper introduces three NBCCS patients and describes their symptoms. In our first case, two major (multiple BCCs in younger than 30 years of age, and palmar pits) and one minor (medulloblastoma) criteria were met, which were indicative of Gorlin syndrome. The second case had two major criteria (multiple BCCs in younger than 30 years of age and odontogenic keratocyst), which confirmed Gorlin syndrome. The third case had one major criterion (multiple BCCs) and two minor criteria (congenital skeletal anomaly and frontal bossing) which confirmed Gorlin syndrome. Early diagnosis and treatment is important in preventing long term squeals of this syndrome that include malignancy and oromaxillofacial deformation and destruction.
کلیدواژه‌ها [English]
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