Clinical Spectrum of Congenital Ichthyosis in a Moroccan Pediatric Study

Abstract

Background: Congenital ichthyoses are rare genetic skin disorders characterized by abnormal keratinization, often present at birth and associated with various extracutaneous manifestations. Material and Methods: We conducted a retrospective 30-month study at a Moroccan university hospital to evaluate clinical presentations of congenital ichthyosis in 40 pediatric patients. The aim was to describe their clinical characteristics and associated findings in order to improve early recognition and care strategies. Results: Forty patients were included (mean age: 3 years; 25 females, 15 males). Lamellar ichthyosis was the most common form (28 cases), with generalized scaling in 80% and palmoplantar keratoderma and nail dystrophy in 50%. Ectropion was observed in 8 patients. Netherton syndrome was identified in 6 patients, with typical pruritic erythroderma and bamboo hair in 3 cases. 2 neonates had harlequin ichthyosis with severe ectropion, eclabium, and one case of limb hypoplasia. Conclusion: Lamellar ichthyosis was the most common subtype. Netherton syndrome and harlequin ichthyosis were also identified. Our findings emphasize the need for early diagnosis, trichoscopic evaluation, and multidisciplinary management in resource-limited settings.