History:

This 4 month old infant born of consanguineous marriage prsented with a few month history of developing a generalized eruption comprised of erythematous, polycyclic, serpiginous plaques.  Scalp was also affected with sparse hairs.         Netherton syndrome is a rare disorder inherited in an autosomal recessive pattern consisting of ichthyosiform dermatosis, hair shaft abnormalities (trichorrhexis invaginata), and an atopic diathesis.

Editor's Comments This is a great example of the double edged polycyclic scaly rash seen in this condition but it often only occurs after the age of 2. The child is born erythrodermic and may be misdiagnosed as congenital ichthyosiform erythroderma. Sparse hair will show the ball in socket deformity of bamboo hair (trichorrhexis invaginata).