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Most commonly occurring on the head and neck, fibrofolliculomas appear as white/gray or yellow papules. Multiple fibrofolliculomas appearing on the face in young adults are associated with Birt-Hogg-Dubé syndrome (BHD). BHD is an autosomal dominant condition caused by germline mutations in the folliculin gene (17p11.2). Other manifestations of the disease include bilateral pulmonary cysts, spontaneous pneumothorax, chromophobe and hybrid chromophobe/oncocytic renal cell tumors.

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