ABSTRACT
Birt-Hogg-Dubè syndrome (BHDS) is a rare autosomal dominant disease, characterized by internal manifestations (such as visceral malignancies, pulmonary cysts and recurrent pneumothorax) and cutaneous lesions. Fibrofolliculomas (FFs) and trichodiscomas (TDs) are the main skin manifestations of BHDS, and recognition of these cutaneous lesions could determine a rapid diagnosis of this rare syndrome and the relative associated complications. Dermoscopically, FF is mainly characterized by the presence of a whitish homogeneous pattern, with typical follicular openings. Recently, a new skin lesion associated with BHDS has been described, named angiomatous nodules. However, the low number of cases present in the literature limits the reproducibility of these dermoscopic features in daily clinical practice.
