gms | German Medical Science

Background and state of research: Patients with hereditary ichthyosis permanently suffer from scaling due to mutations affecting the epidermal barrier. Symptomatic lifelong treatment with ointments, bathing, and mechanical desquamation can alleviate the condition, but therapy requires time and money and is supposed to have a high impact on quality of life of the patients and their families.

Research question and objectives, hypothesis: The study aimed at analyzing the humanistic burden of patients with hereditary ichthyosis with a special regard to financial, personal and time burden.

Method: Within the cross-sectional study 144 patients with ichthyosis including common, rare and syndromic subtypes (mean age: 23; 53.5% female) were recruited in a university-based setting in Germany and via the German support group. A questionnaire to assess the time, personal and financial burden of treatment was developed. Disease classification and severity were assessed by trained physicians. Quality of life impairment was analyzed with the Dermatology Life Quality Index (DLQI) and the Pruritus Life Quality (5PLQ) questionnaire. Difficulties of medical care for ichthyosis were addressed and opportunities for improvement identified.

Results: Eighty-seven percent of respondents reported to use topical medications at least once daily, 66.4% more than once. The median time spent for skin care was 15 minutes per day. Balneotherapy accounted for the highest single time expenditure (n=115; median bath duration: 40 minutes).

In 81.9%, health insurance did not fully cover the cost of topical treatment, resulting in an additional financial burden, with a median of €71 per quarter and creams being the largest cost factor (€50). Patients with Netherton syndrome had the highest median expenditure (€170). The severity of ichthyosis as well as the subtype and frequency of topical therapy revealed a significant influence on the cost in multivariate analysis (p < 0,001). Quality of life impairment during treatment was moderate (median DLQI: 8.5 points). Pruritus was present in 79.9% of patients and had a distinct impact on quality of life (median 5PLQ: 7.5 points), with no significant difference between subtypes (p=0.37).

Discussion: Patients suffering from ichthyosis have a large and lifelong overall burden in both mild and severe subtypes. Time effort for topical treatment and bathing is enormous and results in high out-of-pocket cost. Since continuous intensive treatment is required, financial and psychosocial support must be considered in addition to standard dermatological care.

Implication for care: Effective outpatient medical management and financial support of patients with inherited ichthyosis is necessary to alleviate humanistic burden.

Funding: Other funding; SÜ212007