How much disability is caused by fibrofolliculomas during Birt-Hogg-Dubé Syndrome?

J Eur Acad Dermatol Venereol. 2011 Aug;25(8):940-4. doi: 10.1111/j.1468-3083.2010.03887.x. Epub 2010 Nov 4.

Abstract

Background: Facial fibrofolliculomas (FFs) develop mainly on the face of patients with Birt-Hogg-Dubé syndrome (BHDS) after the second decade and increase in number and size during life.

Objectives: Measurement of the impairment of health-related quality of life (HQoL) related to facial FFs in a series of French patients with BHDS in an analytical epidemiology study using two self-reported HQoL questionnaires.

Study design: The Dermatology Life Quality Index© (DLQI) and a modified version of the validated French version of the Cardiff Acne Disability Index© (CADI) were sent to 19 patients with BHDS. Demographic and clinical data were collected.

Analysis: Statistical analysis was performed using SAS version 9.

Results: Eighteen patients (56% male, 44% female, mean age 50.2 years) responded. Self-reported alteration in HQoL was reported in approximately one-third of the patients. The mean DLQI score (maximum 30) was low 2.2 (±4.3, range 0-18). Six (33.3%) had small effect on life (score 2-5) and one patient (5.5%) had a very large effect on her life (score 11-20) scoring 18. The mean CADI score (maximum 15) was a low 3.3 (±4.36, range 0-13). Five patients (31.1%) scored over 5 (>33% impairment). The highest DLQI and CADI scores were related to higher number of FFs. Limitation relies on the absence of validation of the modified version of the CADI.

Conclusions: This study stresses the burden of facial FFs for a minority of patients and the importance of providing a local treatment.

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Birt-Hogg-Dube Syndrome / psychology*
  • Facial Neoplasms / psychology*
  • Female
  • Hair Diseases / psychology
  • Hair Follicle*
  • Humans
  • Male
  • Middle Aged
  • Quality of Life / psychology*
  • Self Report
  • Young Adult