The department of Dermatology at the American University of Beirut Medical Center is a referral center for genodermatoses all over Lebanon and the Middle East region. We recruited 25 Middle Eastern patients with Ichthyosis and Ichthyosiform disorders, 17 of whom were a product of consanguineous marriage. We included all patients no matter their age, gender, past or current treatments, or type of Ichthyosis. Pregnant females were excluded. Many of our patients were using emollients and creams containing variable concentrations of urea and glycolic acid with suboptimal responses. All of these topical therapies were discontinued at the study onset. One patient was on oral isotretinoin therapy. Within one week of introducing our topical creams, he developed severe irritation which only improved slightly upon altering the frequency of application. He was therefore excluded from the study. The study was approved by the institutional review board at the American University of Beirut, and all patients provided their written informed consent.
Treatment creams were applied on specific areas chosen by both the patient and the physician. The combination cream of lovastatin, 2%- cholesterol, 2% was applied every morning. A glycerol-based moisturizer was applied every afternoon. The combination cream of glycolic acid, 10%- tretinoin, 0.025%- ketoconazole, 2% was applied nightly or every other night depending on tolerability.
Patients were scheduled for monthly visits, during which scoring was done and pictures were taken. The primary outcome was clinical improvement after 2 and 3 months of treatment. Some patients continued treatment beyond 3 months. Unfortunately, because of the Lebanese revolution which started in October 2019 and the COVID-19 pandemic of 2020, some patients were not able to visit us every month. However, all patients were able to come to at least one follow-up visit.
Scoring System
We used the same severity scoring system used in our previous study.2 Five different criteria were assessed during each visit: (1) skin thickness and/or scaling; (2) pain, pruritus, and/or discomfort; (3) erythema; (4) impact of the condition on the ability to perform daily functions; and (5) the patient’s subjective severity scoring, ie, the patient’s own assessment of the severity of his/her condition. Each criterion was given a score from 0 to 20, 0 being absent and 20 being most severe. (Table I). For criterion number (4) or the impact of the condition on the ability to perform daily functions, the Dermatology Life Quality Index (DLQI) was used. A score out of 30 was obtained and was converted to a score out of 20. For small children, the questions were asked to the parents whenever applicable (eg. Over the last month, how often do you see your child itching his skin lesions?).
The degree of improvement at follow-up was assessed by the percentage reduction in the total severity score compared to baseline. A reduction of 0–9.9% was considered a poor response; 10.0–24.9%, a fair response; 25.0–49.9%, good response; 50–74.9%, very good response; and 75–100%, excellent response.
The primary outcomes were the changes in the average severity scores (compared to baseline) at 1, 2 and 3 months of follow-up. The secondary outcomes were the changes in the average severity scores (compared to baseline) at 4, 5 and 6 months of follow-up, and the rates of adverse events.