Quality of life among pediatric patients and their families suffering from congenital ichthyosis - A cross-sectional study

Abstract

Introduction: Ichthyosis are a heterogeneous group of hereditary skin disorders characterized by dryness, hyperkeratosis, and desquamation. The purpose of this study was to evaluate quality of life (QoL) of patients with ichthyosis and their families. Materials and Methods: A total of 72 patients were enrolled and 60 patients completed the study. The study instrument included Infants' Dermatitis Life Quality Index and Children's Dermatology Life Quality Index for pediatric patients (5–16 years) and Dermatitis Family Impact Questionnaire (DFI) for family members. Results: Questionnaires were completed in 60 children. The patients had predominantly ichthyosis vulgaris (n = 46), lamellar ichthyosis (n = 10), nonbullous ichthyosiform erythroderma (n = 3), and bullous ichthyosiform erythroderma (n = 1). Male: female ratio was 1:1.5. Large effect on QoL was seen in 77.8% and 66.6% of ichthyosis vulgaris and lamellar, ichthyosis, respectively. Mean score of DFI was 9.94 ± 7.23. Conclusion: The present study reports a poor QoL among pediatric patients suffering from ichthyosis and their families. Psychosocial and mental support is needed for patients and their immediate caregivers/families for this chronic skin disease.

Keywords

• dermatitis family impact
• ichthyosis
• quality of life
• support