Neonatal and infantile erythroderma is a diagnostic and therapeutic challenge. Numerous underlying causes have been reported. Etiologic diagnosis of erythroderma is frequently difficult to establish, and is usually delayed, due to the poor specificity of clinical and histopathologic signs. Differential diagnosis of erythroderma is a multi-step procedure that involves clinical assessment, knowledge of any relevant family history and certain laboratory investigations. Immunodeficiency must be inspected in cases of severe erythroderma with alopecia, failure to thrive, infectious complications, or evocative histologic findings. The prognosis is poor with a high mortality rate in immunodeficiency disorders and severe chronic diseases such as Netherton's syndrome.
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