Patient demographic characteristics and tumor-specific features may provide predictive information regarding the risk of postsurgical recurrence and metastasis after treatment of PCMC.

A reliable method for assessing ichthyosis severity has not been uniformly agreed upon. The objective of our study was to develop and validate a tool to measure the clinical severity of the congenital ichthyoses, the Congenital Ichthyoses Severity Index. A prospective study was performed to determine reliability of three Likert scales to evaluate clinical severity of ichthyosis. Thirty-eight subjects recruited from the Foundation for Ichthyosis and Related Skin Types National Conference were evaluated separately by two blinded investigators using the Likert scales. Subjects were then asked to evaluate themselves using these scales. Inter-rater reliability was determined between ratings provided by all three raters. Test-retest validation was conducted with 21 subjects who completed follow-up surveys 4 weeks later. Our severity scales had excellent inter-rater and test-retest reliability as determined by intraclass correlation coefficients (ICC >0.7), with the exception of our hyperkeratosis scale, which demonstrated moderate test-retest reliability (ICC = 0.4). This pilot study provides a promising method for evaluating clinical severity of the congenital ichthyoses, one easily employed by both physicians and patients. Future epidemiologic studies may benefit from use of this instrument, as well as studies evaluating emerging therapies for ichthyosis.

We explored resource utilization (ResUtil) and quality of life (QOL) associated with congenital ichthyoses (CI). Subjects completed an online survey related to clinical severity, demographics, ResUtil, and QOL as measured according to the Dermatology Life Quality Index (DLQI). Validated Likert scales were used to evaluate severity of hyperkeratosis, erythema, and alopecia. ResUtil was determined according to time spent daily treating CI symptoms (TimeTx) and number of ichthyosis-related dermatology visits (DermVisits) per year. We used linear regression to investigate predictors of a transformed DLQI (sqrtDLQI) and logistic regression for ResUtil. Of 235 subjects, 60.2% were female, 83.8% were Caucasian, 42.3% had a family history (FamHx) of CI, and the mean age was 28.7 years (SD 20.3). Predictors for worse QOL were hyperkeratosis severity (β = 0.27, p < 0.01), erythema (β = 0.27, p < 0.01), TimeTx (β = 0.21, p < 0.01), ichthyosis type (β = 0.09, p < 0.01), and age (β = 0.01, p = 0.02). Predictors for DermVisits were hyperkeratosis severity (odds ratio [OR] = 1.38, 95% confidence limit [CL] = 1.01, 1.87), FamHx (OR = 0.28, 95% CL = 0.09, 0.85), age (OR = 0.97, 95% CI = 0.96, 0.99), and alopecia severity (OR = 1.43, 95% CL = 1.12, 1.82). Predictors for treatment duration were erythema (OR = 1.35, 95% CL = 1.02, 1.78), age (OR = 0.98, 95% CL = 0.96, 0.99), and DLQI (OR = 1.09, 95% CL = 1.03, 1.15). Increased hyperkeratosis severity and erythema negatively impact QOL in the CI. Furthermore, increased disease severity predicted greater ResUtil, whereas increased age and FamHx predicted less ResUtil. Our findings suggest that better therapies and increased patient education may improve QOL and decrease ResUtil.

The aim of this study is to estimate annual, per patient, health care costs for congenital ichthyoses (CI). We conducted a cost analysis through an online survey posted on the Foundation for Ichthyosis and Related Skin Types Website. We assessed cutaneous disease severity, via the previously validated Congenital Ichthyosis Severity Index (CISI), demographics, and CI type. We estimated direct health care costs: prescription and over-the-counter medications, outpatient visits, and emergency department and hospital visit costs; and indirect costs: earnings lost owing to absences from work because of CI-related illness. The CI subjects of our study (n=224) consumed a mean (SD) of $3,192 ($7,915) annually. Direct costs accounted for 90%, whereas indirect costs accounted for 10%. These costs resulted in an estimated annual cost of $37 MM/year (excluding ichthyosis vulgaris) of which $17 MM is borne out-of-pocket by patients. Depending on the CI diagnosis, patients were responsible for 30-51 cents of every dollar of mean annual medical care costs. Our estimated annual CI costs are comparable to cutaneous lymphoma. More effective treatments for CI would help minimize this burden. Traditional insurance products do not appear to substantially alleviate the financial burden of disease, as a significant amount is from out-of-pocket expenses.