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Fibrofolliculoma

MedGen UID:
138032
•Concept ID:
C0346011
•
Neoplastic Process
Synonym: Fibrofolliculomas
SNOMED CT: Fibrofolliculoma (128683008); Follicular fibroma (128683008); Fibrofolliculoma (254699007)
 
HPO: HP:0030436

Definition

Fibrofolliculoma is a clinically asymptomatic, 2-4 mm, skin-colored, dome-shaped smooth papule. It usually arises in the form of multiple lesions in adults in different areas such as the scalp, forehead, face, and neck. According to histology, the lesion is a fibrotic hamartoma characterized by infundibular epithelial proliferation and perifollicular fibrous proliferation. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVFibrofolliculoma

Conditions with this feature

Birt-Hogg-Dube syndrome 2
MedGen UID:
1841312
•Concept ID:
C5830676
•
Disease or Syndrome
Birt-Hogg-Dube syndrome-2 (BHD2) is characterized by lipomatosis and fibrofolliculomas as well as renal cell carcinoma and other cancers (van de Beek et al., 2023). For a general phenotypic description and a discussion of genetic heterogeneity of BHD, see BHD1 (135150).
See: Condition Record
Birt-Hogg-Dube syndrome 1
MedGen UID:
1051978
•Concept ID:
CN375946
•
Disease or Syndrome
Any Birt-Hogg-Dube (BHD) syndrome in which the cause of the disease is a variation in the FLCN gene.
See: Condition Record
Birt-Hogg-Dube syndrome 1
Birt-Hogg-Dube syndrome 2

Professional guidelines

PubMed

Birt-Hogg-Dubé syndrome: a review of the literature and the differential diagnosis of firm facial papules.
Vincent A, Farley M, Chan E, James WD
J Am Acad Dermatol 2003 Oct;49(4):698-705. doi: 10.1067/s0190-9622(03)01582-2. PMID: 14512919

Recent clinical studies

Etiology

What is in a name-Perifollicular fibroma or fibrofolliculoma?
Haneke E, Happle R
J Cutan Pathol 2024 Feb;51(2):170-175. Epub 2023 Aug 30 doi: 10.1111/cup.14522. PMID: 37649357
Solitary fibrofolliculoma: a retrospective case series review over 18 years.
Díez-Montero C, Alonso MD, Marquez PIG, Schellini SA, Galindo-Ferreiro A
Arq Bras Oftalmol 2020 Nov-Dec;83(6):485-489. doi: 10.5935/0004-2749.20200092. PMID: 33470275
Benign follicular tumors.
Tellechea O, Cardoso JC, Reis JP, Ramos L, Gameiro AR, Coutinho I, Baptista AP
An Bras Dermatol 2015 Nov-Dec;90(6):780-96; quiz 797-8. doi: 10.1590/abd1806-4841.20154114. PMID: 26734858Free PMC Article
Birt-Hogg-Dubé syndrome and the skin.
Vernooij M, Claessens T, Luijten M, van Steensel MA, Coull BJ
Fam Cancer 2013 Sep;12(3):381-5. doi: 10.1007/s10689-013-9600-8. PMID: 23307118
Association between Birt Hogg Dube syndrome and cancer predisposition.
Palmirotta R, Savonarola A, Ludovici G, Donati P, Cavaliere F, DE Marchis ML, Ferroni P, Guadagni F
Anticancer Res 2010 Mar;30(3):751-7. PMID: 20392993

Diagnosis

Solitary fibrofolliculoma: a retrospective case series review over 18 years.
Díez-Montero C, Alonso MD, Marquez PIG, Schellini SA, Galindo-Ferreiro A
Arq Bras Oftalmol 2020 Nov-Dec;83(6):485-489. doi: 10.5935/0004-2749.20200092. PMID: 33470275
Benign follicular tumors.
Tellechea O, Cardoso JC, Reis JP, Ramos L, Gameiro AR, Coutinho I, Baptista AP
An Bras Dermatol 2015 Nov-Dec;90(6):780-96; quiz 797-8. doi: 10.1590/abd1806-4841.20154114. PMID: 26734858Free PMC Article
Birt-Hogg-Dubé syndrome and the skin.
Vernooij M, Claessens T, Luijten M, van Steensel MA, Coull BJ
Fam Cancer 2013 Sep;12(3):381-5. doi: 10.1007/s10689-013-9600-8. PMID: 23307118
A solitary fibrofolliculoma in the eyelid.
Chang JK, Lee DC, Chang MH
Korean J Ophthalmol 2007 Sep;21(3):169-71. doi: 10.3341/kjo.2007.21.3.169. PMID: 17804924Free PMC Article
Birt-Hogg-Dubé Syndrome.
Welsch MJ, Krunic A, Medenica MM
Int J Dermatol 2005 Aug;44(8):668-73. doi: 10.1111/j.1365-4632.2004.02095.x. PMID: 16101870

Therapy

Birt-Hogg-Dubé syndrome: Clinical and molecular aspects of recently identified kidney cancer syndrome.
Hasumi H, Baba M, Hasumi Y, Furuya M, Yao M
Int J Urol 2016 Mar;23(3):204-10. Epub 2015 Nov 25 doi: 10.1111/iju.13015. PMID: 26608100
Topical rapamycin as a treatment for fibrofolliculomas in Birt-Hogg-Dubé syndrome: a double-blind placebo-controlled randomized split-face trial.
Gijezen LM, Vernooij M, Martens H, Oduber CE, Henquet CJ, Starink TM, Prins MH, Menko FH, Nelemans PJ, van Steensel MA
PLoS One 2014;9(6):e99071. Epub 2014 Jun 9 doi: 10.1371/journal.pone.0099071. PMID: 24910976Free PMC Article

Prognosis

Epidemiology and clinical features of Birt-Hogg-Dubé syndrome: A nationwide population-based study in South Korea.
Park HJ, Kim YJ, Kim MJ, Kim HC
PLoS One 2022;17(6):e0269358. Epub 2022 Jun 6 doi: 10.1371/journal.pone.0269358. PMID: 35666727Free PMC Article
Colorectal cancer risk in families with Birt-Hogg-Dubé syndrome increased.
Sattler EC, Syunyaeva Z, Reithmair M, Dempke W, Steinlein OK
Eur J Cancer 2021 Jul;151:168-174. Epub 2021 May 14 doi: 10.1016/j.ejca.2021.04.013. PMID: 34000505
Solitary fibrofolliculoma: a retrospective case series review over 18 years.
Díez-Montero C, Alonso MD, Marquez PIG, Schellini SA, Galindo-Ferreiro A
Arq Bras Oftalmol 2020 Nov-Dec;83(6):485-489. doi: 10.5935/0004-2749.20200092. PMID: 33470275
Birt-Hogg-Dubé syndrome: Clinical and molecular aspects of recently identified kidney cancer syndrome.
Hasumi H, Baba M, Hasumi Y, Furuya M, Yao M
Int J Urol 2016 Mar;23(3):204-10. Epub 2015 Nov 25 doi: 10.1111/iju.13015. PMID: 26608100
Review of renal oncocytosis (multiple oncocytic lesions) with focus on clinical and pathobiological aspects.
Kuroda N, Tanaka A, Ohe C, Mikami S, Nagashima Y, Sasaki T, Inoue K, Hes O, Michal M, Brunelli M, Martignoni G
Histol Histopathol 2012 Nov;27(11):1407-12. doi: 10.14670/HH-27.1407. PMID: 23018240

Clinical prediction guides

Solitary asymptomatic lobular perianal nodule on an infant.
Omorodion I, Lee J, Gru AA, Zlotoff BJ
Dermatol Online J 2021 Oct 15;27(10) doi: 10.5070/D3271055628. PMID: 35130389
Colorectal cancer risk in families with Birt-Hogg-Dubé syndrome increased.
Sattler EC, Syunyaeva Z, Reithmair M, Dempke W, Steinlein OK
Eur J Cancer 2021 Jul;151:168-174. Epub 2021 May 14 doi: 10.1016/j.ejca.2021.04.013. PMID: 34000505
Histopathological features of fibrous cephalic plaques in tuberous sclerosis complex.
Treichel AM, Pithadia DJ, Lee CR, Oyerinde O, Moss J, Darling TN
Histopathology 2021 Oct;79(4):619-628. Epub 2021 Jul 21 doi: 10.1111/his.14392. PMID: 33882161Free PMC Article
Solitary fibrofolliculoma: a retrospective case series review over 18 years.
Díez-Montero C, Alonso MD, Marquez PIG, Schellini SA, Galindo-Ferreiro A
Arq Bras Oftalmol 2020 Nov-Dec;83(6):485-489. doi: 10.5935/0004-2749.20200092. PMID: 33470275
Fibrofolliculoma in a patient with tuberous sclerosis complex.
Misago N, Narisawa Y
Clin Exp Dermatol 2009 Dec;34(8):892-4. Epub 2009 Jan 15 doi: 10.1111/j.1365-2230.2008.03065.x. PMID: 19196303

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