title: Quality of life in adults with congenital ichthyosis.
abstract: BACKGROUND
  Little is known about the quality of life of people with congenital and generalized skin diseases. Describing life history and quality of life from an individual perspective could increase understanding of living with generalized congenital ichthyosis for nursing staff and others.
  
  
  OBJECTIVE
  The aim of our study was to illustrate how middle-aged and older people with lamellar ichthyosis or epidermolytic hyperkeratosis describe the effects of their disease on their quality of life.
  
  
  METHODS
  Six women and four men aged 56-80 years participated. A global clinical assessment of the severity of the disease was performed. The Nottingham Health Profile (NHP) questionnaire was used to investigate quality of life. Participants were interviewed face-to-face about childhood and adulthood experiences of living with a skin disease. Interview data were analysed using content analysis.
  
  
  RESULTS
  Interview data were assigned to two categories, childhood and adulthood, and organized under 16 themes. All interviewees reported that their skin disease had affected them negatively to varying degrees during their entire lives, and that the most problematic period was childhood. Coping strategies used during childhood were hiding of the skin and developing shyness. There were no correlations between objective signs of ichthyosis and the NHP scores.
  
  
  CONCLUSIONS
  Congenital ichthyosis appears to affect several aspects of life negatively, and it is hoped that an understanding of the effects of the disease will lead to more efficient nursing care.
authors.affiliation: Department of Medical Sciences, Section of Dermatology, Uppsala University, Uppsala, Sweden. ab.ganemo@lm.lrf.se
authors.forename: Agneta
authors.initials: A
authors.lastname: Gånemo
authors.forename: Christina
authors.initials: C
authors.lastname: Lindholm
authors.forename: Magnus
authors.initials: M
authors.lastname: Lindberg
authors.forename: Per-Olow
authors.initials: PO
authors.lastname: Sjödén
authors.forename: Anders
authors.initials: A
authors.lastname: Vahlquist
mesh_terms: D000223:Adaptation, Psychological; D000368:Aged; D000369:Aged, 80 and over; D001294:Attitude to Health; D005260:Female; D006801:Humans; D007057:Ichthyosis / Q000523:psychology*; D007398:Interpersonal Relations; D008297:Male; D008875:Middle Aged; D011788:Quality of Life; D011795:Surveys and Questionnaires