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J Cutan Pathol. 2012 Jul;39(7):675-9. doi: 10.1111/j.1600-0560.2012.01929.x.

Perifollicular fibroma in Birt-Hogg-Dubé syndrome: an association revisited.

Author information

1
Department of Pathology, New York University School of Medicine, New York, NY 10016, USA. Marianna.Shvartsbeyn@nyumc.org

Abstract

BACKGROUND:

Mutation in the folliculin gene in Birt-Hogg-Dubé (BHD) syndrome leads to a spectrum of benign tumors of the hair follicle, classically including both fibrofolliculoma and trichodiscoma. In addition, lesions clinically indistinguishable from fibrofolliculoma/ trichodiscoma may show histopathologic findings of perifollicular fibroma or angiofibroma. Although some consider perifollicular fibroma to be a variant of angiofibroma, the specific histopathologic findings of perifollicular fibroma are uncommon.

METHODS:

This is a case series.

RESULTS:

In 4 patients with multiple facial lesions, recognizing perifollicular fibroma would have been helpful in leading to the diagnosis of BHD syndrome.

CONCLUSION:

Perifollicular fibroma is on a spectrum with fibrofolliculoma and trichodiscoma; in a patient with multiple lesions, the diagnosis of perifollicular fibroma is suggestive of BHD syndrome.

[Indexed for MEDLINE]

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