Skin-colored papules on the cheeks, acrochordons on the axillae.

Abstract

A 43-year-old woman presented to the dermatology department with multiple small skin-colored papules on her cheeks (Figure 1) and acrochordons (“skin tags”) on the axillae (Figure 2). She reported that they had appeared over the past few years. Her personal medical history was unremarkable, but she had a fi rst-degree relative with renal carcinoma. Biopsy of a papule revealed dermal follicular structures surrounded by a perifollicular fi brous sheath and a densely fi brous stroma, consistent with a fi brofolliculoma (Figure 3), ie, a benign hair follicle tumor pathognomonic for Birt-HoggDubé (BHD) syndrome. Subsequent genetic testing found a mutation in the FLCN gene, confi rming the diagnosis. Computed tomography showed no evidence of lung cysts or kidney tumor. Regular cancer surveillance was instituted.