Netherton Syndrome

A 17-year-old Egyptian patient with Netherton syndrome presented with pruritic dry skin and short brittle hair since infancy. Born to healthy nonconsanguineous parents, he developed erythroderma and scaling at 4 weeks old, along with recurrent infections and failure to thrive in his first year. By age 2, his growth normalized, but he developed erythematous, scaly lesions. Despite these symptoms, he achieved normal developmental milestones and intelligence. Family history included eczema and allergic rhinitis but not ichthyotic disorders. Physical examination revealed scaly, hyperkeratotic lesions and sparse, brittle hair with bamboo hair characteristics. Elevated IgE levels were noted, and a skin biopsy confirmed the diagnosis. Treatment included antihistamines, emollients, and 10% urea.