Lamellar ichthyosis (LI) is an autosomal recessive disorder that is apparent at birth and is present throughout life. The newborn is born encased in a collodion membrane that sheds within 10-14 days. The shedding of the membrane reveals generalized scaling with variable redness of the skin. The scaling may be fine or platelike, resembling fish skin (formation of large, coarse scales involving the entire body, including all flexural areas as well as the palms and soles). Although the disorder is not life threatening, it is quite disfiguring and causes considerable psychological stress to affected patients.
Collodion baby-shortly after birth with a parchment-like membrane covering the entire skin. The eyes and lips pucker outward, i.e., ectropion and eclabion.
Same baby as above at 6 months of age with minimal residual scale and erythema on the cheeks.
Large parchment like scales over the entire body. Mild ectropion was also present in this patient.
Body distribution of lamellar ichthyosis.
Parchment-like hyperkeratosis of lamellar ichthyosis gives the impression of the skin being too tight on the face of this woman. She has lost all hairs and there is pronounced ectropium.
Lamellar ichthyosis: armsTesselated (tile-like) hyperkeratosis gives the appearance of reptilian scales on the arms around the elbows. The entire body was involved and there was ectropium.