Hair Follicle Neoplasms - Birt Hogg Dube Syndrome

Birt Hogg Dube Syndrome is a genetic syndrome that I rarely encounter in both my The Woodlands dermatology and Conroe dermatology clinics.  It is due to a genetic mutation in the gene folliculin.  Patients with Birt Hogg Dube manifests three benign hair follicle neoplasms: Fibrofolliculomas, Trichodiscomas, and Perifollicular Fibromas.  Clinically, all three of these lesions have a similar appearance in that they are small flesh colored papules arising on the face and upper chest/neck.  Patients with Birt Hogg Dube Syndrome are at increased risk of developing renal tumors and spontaneous pneumothorax (the lung collapses).  The Fibrofolliculomas, Trichodiscomas and Perifollicular Fibromas do not need to be treated unless the patient desires and shave excision is the most aesthetically pleasing method of removal.

Fibrofolliculomas, Trichodiscomas, Perifollicular Fibromas (http://atlasgeneticsoncology.org/Kprones/Images/BHDSFig1.jpg)

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