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.
Aim. 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.