Netherton Syndrome

ABSTRACT This case describes a 9-day-old female infant who presented with severe skin peeling and diffuse erythema. As the patient was refractory to standard treatment, a working differential diagnosis was built and included infectious, inflammatory, and inherited etiologies. The patient’s workup pointed toward Netherton syndrome, a rare autosomal recessive condition that presents at birth or within the first few weeks of life and is characterized by widespread erythroderma with ichthyosis.