Intérêt de la recherche de Lekti dans le diagnostic d'une érythrodermie chez le nourrisson

Netherton syndrome is a rare genodermatosis characterized by ichthyosiform scaling, hair shaft abnormalities and atopic features. We report two cases of Netherton syndrome in an 18-month-old girl and a 24-month-old boy. The girl presented pruriginous erythroderma, frequent otitis, growth retardation and Trichorrhexis invaginata. Immunohistochemistry shows LEKTI deficiency. The boy presented non-bullous congenital erythroderma, signs of atopy, several episodes of bacterial infection and growth retardation. The hair was normal; immunohistochemistry showed LEKTI deficiency. Immunohistochemistry of LEKTI protein allows a fast diagnosis of Netherton syndrome.