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J Cutan Pathol. 2002 Oct;29(9):529-33.

Histomorphologic and immunophenotypic analysis of fibrofolliculomas and trichodiscomas in Birt-Hogg-Dube syndrome and sporadic disease.

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  • 1Department of Pathology, University of South Florida College of Medicine, Tampa, FL, USA. gcollins@hsc.usf.edu

Abstract

BACKGROUND:

Fibrofolliculomas and trichodiscomas are benign dermal neoplasms that likely derive from the mantle of the hair follicle and can occur sporadically or in association with Birt-Hogg-Dube syndrome (BHDS). Little is known about the pathogenesis and immunophenotypic properties of these entities.

METHODS:

We investigated the histomorphologic and immnophenotypic properties of 15 fibrofolliculomas and trichodiscomas in two patients with BHDS and eight with sporadic disease.

RESULTS:

We found the following: (1) fibrofolliculomas and trichodiscomas occurring in the setting of BHDS show microscopically contiguous histomorphologic features as compared to those occurring sporadically; (2) the immunophenotypic characteristics of syndromic-associated and sporadic types are identical and consist of (3) perifollicular vimentin (+), CD34 (+) and Factor XIII (-) spindle cells.

CONCLUSIONS:

Despite subtle histomorphologic differences, trichodiscomas and fibrofolliculomas are immunophenotypically similar, and are thus likely derived from a similar histogenic precursor. Given the previously reported CD34 (+) immunophenotype of the hair mantle, our findings would support an origin of these lesions from the mantle of the hair follicle. The proliferation of CD34 (+) spindle cells seen in conjunction with these lesions should not be confused with other CD34 (+) dermal entities.

PMID:
12358810
[PubMed - indexed for MEDLINE]
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