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

J Cutan Pathol. 2002 Oct;29(9):529-33. doi: 10.1034/j.1600-0560.2002.290904.x.


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.

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Biomarkers, Tumor / analysis
  • Female
  • Fibroma / chemistry
  • Fibroma / pathology*
  • Hair Diseases
  • Hair Follicle / chemistry
  • Hair Follicle / pathology
  • Humans
  • Immunohistochemistry
  • Immunophenotyping
  • Male
  • Middle Aged
  • Neoplasm Proteins / analysis
  • Neoplasms, Multiple Primary / chemistry
  • Neoplasms, Multiple Primary / pathology*
  • Skin Neoplasms / chemistry
  • Skin Neoplasms / pathology*
  • Syndrome


  • Biomarkers, Tumor
  • Neoplasm Proteins