Birt Hogg Dube syndrome

An inherited condition in which benign tumors develop in hair follicles on the head, chest, back, and arms. These tumors are called fibrofolliculomas. Other characteristic skin abnormalities are trichodiscomas and acrochordons. A trichodiscoma is a tumor of the hair disc while an acrochordon is a skin tag. People with this syndrome are at increased risk for developing colon or kidney cancer as well as spontaneous pneumothorax due to lung cysts.

The Birt-Hogg-Dube syndrome is inherited in an autosomal dominant manner and is due to mutation in the gene on chromosome 17 encoding a protein called folliculin. The disorder is also known as Birt Hogg Dube syndrome, Birt-Hogg-Dube syndrome, and fibrofolliculomas with trichodiscomas and acrochordons.