Nevoid basal cell carcinoma syndrome: our experience in a pediatric hospital.
نویسندگان
چکیده
Nevoid basal cell carcinoma (BCC) syndrome, or Gorlin syndrome, is a rare autosomal dominant disorder associated with mutations in the patched 1 gene, PTCH1. It is characterized by the presence of multiple BCCs in association with disorders affecting the bones, the skin, the eyes, and the nervous system. We describe 6 cases of nevoid BCC syndrome evaluated in our department. Palmoplantar pitting was observed in all 6 patients, multiple BCCs in 5 patients (83%), skeletal anomalies in 3 patients (50%), and odontogenic keratocysts in 1 patient (17%). We would like to stress the importance of early diagnosis and treatment in nevoid BCC syndrome and the need for continuous, long-term follow-up by a multidisciplinary team.
منابع مشابه
Odontogenic keratocysts in a 5-year-old: initial manifestations of nevoid basal cell carcinoma syndrome.
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ورودعنوان ژورنال:
- Actas dermo-sifiliograficas
دوره 104 5 شماره
صفحات -
تاریخ انتشار 2013