Netherton Syndrome

A 17-year-old Egyptian patient was diagnosed with Netherton syndrome, characterized by pruritic dry skin, short brittle hair, and a history of erythroderma and recurrent infections since infancy. Despite normal developmental milestones and intelligence, the patient exhibited scaly, migratory skin lesions and sparse eyebrows and lashes. Laboratory tests revealed elevated IgE levels, and hair microscopy showed trichorrhexis invaginata, or bamboo hair. Treatment included antihistamines, emollients, and 10% urea. The family history was notable for eczema and allergic rhinitis but not ichthyotic disorders.