27-year-old woman presented with multiple growths on her face and upper body, which she has had for several years. She also reported that her mother and maternal grandmother have similar lesions and that her mother has been diagnosed with Birt-Hogg-Dube syndrome. Histopathologic features of one papule demonstrated basaloid cells emanating from a dilated follicular infundibula surrounded by a well-circumscribed fibromucinous stroma. Birt-Hogg-Dube syndrome is an autosomal-dominant-inherited condition characterized by fibrofolliculomas, trichodiscomas, and acrochordons. It is associated with renal tumors, spontaneous pneumothoraces, and possibly intestinal polyps. Treatment includes evaluation for associated renal, lung, and intestinal findings as well as cosmetic removal of skin lesions.
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