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Skin - Nonmelanocytic tumors

Adnexal tumors-hair follicles

Folliculofibroma

Reviewer: Christopher Hale, M.D. (see Reviewers page)
Revised: 4 July 2012, last major update June 2012
Copyright: (c) 2001-2012, PathologyOutlines.com, Inc.

General
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● Considered a hamartoma of hair follicle

Birt-Hogg-Dubé syndrome
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● Folliculofibroma associated with Birt-Hogg-Dubé (BHD) syndrome
● Autosomal dominant, multiple folliculomas on head and neck, acrocordons and trichodiscomas; also renal cell carcinoma [various types], renal oncocytoma and oncocytic hybrid tumors, lung cysts and spontaneous pneumothorax)
● Human gene at 17p11.2 encodes folliculin, normally expressed in skin and adnexae (Mod Pathol 2004;17:998)
● Frameshift mutations occur in BHD causing premature protein termination

Case reports
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● 54 year old man with papules on cheeks (Ann Dermatol 2011;23:S193)

Clinical images
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Various images

Gross description
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● Multiple skin-colored papules

Micro description
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● Thin epidermal strands originating from a central hair follicle with prominent connective tissue

Micro images
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Various images

Differential diagnosis
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● Trichodiscoma
● Mantleoma
● Perifollicular fibroma
● Trichofolliculoma

End of Skin - Nonmelanocytic tumors > Adnexal tumors-hair follicles > Folliculofibroma

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