Abstract



Netherton syndrome (NS) is an autosomal recessive disease characterized by linear circumflex ichthyosis, atopic diathesis and hair shaft abnormalities. The diagnosis can be complex due to the variability of its presentation. This is a report of a 7-year-old male with generalized dermatosis present since birth; consisting of multiple polycyclic, erythematous and excoriated plaques, covered by serohematic crusts. These lesions presented poorly defined and irregular limits, along with areas of scaly appearance and are intensely itchy.