Lamellar ichthyosis (LI) is a rare skin condition. It appears at birth and continues throughout life.
LI; Collodian baby - lamellar ichthyosis; Ichthyosis congenital; Autosomal recessive congenital ichthyosis - lamellar ichthyosis type
LI is an autosomal recessive disease. This means that the mother and father must both pass one abnormal copy of the disease gene to their child in order for the child to develop the disease.
Many babies with LI are born with a clear, shiny, waxy layer of skin called a collodion membrane. For this reason, these babies are known as collodian babies. The membrane sheds within the first 2 weeks of life. The skin underneath the membrane is red and scaly. It resembles the surface of a fish.
With LI, the outer layer of skin called the epidermis is not normal and cannot protect the body like the healthy epidermis can. As a result, a baby with LI may have the following health problems:
Older children and adults with LI may have these symptoms:
Collodion babies usually need to stay in the neonatal intensive care unit (NICU). They are placed in a high-humidity incubator. They will need extra feedings. Moisturizers need to be applied to the skin. After the collodion membrane is shed, babies can usually go home.
Lifelong care of the skin involves keeping the skin moist to minimize the thickness of the scales. Measures include:
These groups can provide more information about LI:
Babies are at risk for infection when they shed the collodian membrane.
Eye problems may occur later in life because the eyes cannot close completely.
Martin KL. Disorders of keratinization. In: Kliegman RM, Stanton BF, St. Geme JW, Schor NF, eds. Nelson Textbook of Pediatrics. 20th ed. Philadelphia, PA: Elsevier; 2016:chap 658.
Paller AS, Mancini AJ. Hereditary disorders of cornification. In: Paller AS, Mancini AJ, eds. Hurwitz Clinical Pediatric Dermatology. 5th ed. Philadelphia, PA: Elsevier; 2016:chap 5.
Richard G, Ringpfeil F. Ichthyoses, erythrokeratodermas, and related disorders. In: Bolognia JL, Schaffer JV, Cerroni L, eds. Dermatology. 4th ed. Philadelphia, PA: Elsevier; 2018:chap 57.
Data Source : ADAM