Fibrofolliculoma and Trichodiscoma
David Cassarino, MD, PhD
Key Facts
Terminology
Closely related hamartomas with follicular and mesenchymal differentiation
Etiology/Pathogenesis
Multiple lesions are strongly correlated with Birt-Hogg-Dubé syndrome
High risk of renal cell carcinoma
Also, pulmonary cysts and pneumothoraces
Clinical Issues
Small papule(s) on face
Microscopic Pathology
FF shows proliferation of thin, anastomosing epithelial cords
Numerous attachments to follicular structures and epidermis
Typically find a central, dilated follicular structure
TD is a stromal-predominant lesion
 Clinical examination of a patient with Birt-Hogg-Dubé syndrome shows numerous small, flesh-colored papules on the lateral cheek and neck  . (Courtesy J. Wu, MD.) |
 Fibrofolliculoma shows a central dilated (cystic) follicular structure with keratin debris. There are multiple thin, radiating epithelial strands attached to the overlying epidermis  . |
TERMINOLOGY
Abbreviations
Fibrofolliculoma (FF)
Trichodiscoma (TD)
Synonyms
Mantleoma (likely fibrofolliculoma variant with sebaceous differentiation)
Definitions
Closely related hamartomatous proliferations with follicular and mesenchymal differentiation
Trichodiscoma likely represents a late stage in evolution of fibrofolliculoma showing stromal predominance
ETIOLOGY/PATHOGENESIS
Genetic Syndrome
Multiple lesions are strongly correlated with Birt-Hogg-Dubé syndrome (BHD)
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