By Disease Name > Ichthyosis Linearis Circumflexa

Netherton's syndrome

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AKA ichthyosis linearis circumflexa (ILC)
autosomal recessive;  pathogenesis unknown
in a nutshell:  ichthyosis linearis circumflexa and trichorrhexis invaginata

 

clinical:

birth few months:  generalized erythema with scaling with secondary hypernatremia and failure to thrive
later:  migratory, polycyclic, serpiginous plaques with double edged scale along the margins (ichthyosis linearis circumflexa) not "ichthyotic" at all; looks like a superficial blister ("flaky paint")
atopic dermatitis; seborrheic dermatitis
trichorrhexis invaginata (ball and socket configuration, bamboo hair)

 

 

 

see Ichthyoses