• | AKA ichthyosis linearis circumflexa (ILC) |
• | autosomal recessive; pathogenesis – unknown |
• | in a nutshell: ichthyosis linearis circumflexa and trichorrhexis invaginata |
clinical:
• | birth – few month’s: 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
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