Lamellar Ichthyosis Overview
Learn About Lamellar Ichthyosis
Lamellar ichthyosis (LI) is a rare skin condition. It appears at birth and continues throughout life.
LI; Collodion 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 variant 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 collodion babies. The membrane sheds within the first 2 weeks of life. The skin underneath the membrane is red and scaly resembling the surface of a fish.
With LI, the outer layer of skin called the epidermis cannot protect the body like the healthy epidermis can. As a result, a baby with LI may have the following health problems:
- Difficulty in feeding
- Loss of fluid (dehydration)
- Loss of balance of minerals in the body (electrolyte imbalance)
- Breathing problems
- Body temperature that is not stable
- Skin or body-wide infections
Older children and adults with LI may have these symptoms:
- Giant scales that cover most of the body
- Decreased ability to sweat, causing sensitivity to heat
- Hair loss
- Abnormal finger and toenails
- Skin of the palms and soles is thickened
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:
- Moisturizers applied to the skin
- Medicines called retinoids that are taken by mouth in severe cases
- High-humidity environment
- Bathing to loosen scales
Lurie Children's Medical Group Inc.
Amy Paller is a Pediatrics specialist and a Dermatologist in Chicago, Illinois. Dr. Paller and is rated as an Elite provider by MediFind in the treatment of Lamellar Ichthyosis. Her top areas of expertise are Atopic Dermatitis, Nonbullous Congenital Ichthyosiform Erythroderma, Netherton Syndrome, Lamellar Ichthyosis, and Gastrostomy.
Dedee Murrell practices in Kensington, Australia. Murrell and is rated as an Elite expert by MediFind in the treatment of Lamellar Ichthyosis. Their top areas of expertise are Epidermolysis Bullosa, Pemphigus, Pemphigus Vulgaris, and Pemphigus Foliaceus.
Anissa Zaouak practices in Tunis, Tunisia. Ms. Zaouak and is rated as an Elite expert by MediFind in the treatment of Lamellar Ichthyosis. Her top areas of expertise are Nonbullous Congenital Ichthyosiform Erythroderma, Lamellar Ichthyosis, Granuloma Annulare, Benign Chronic Pemphigus, and Vulvectomy.
Babies are at risk for infection when they shed the collodion membrane.
Eye problems may occur later in life because the eyes cannot close completely.
Published Date: October 13, 2024
Published By: Ramin Fathi, MD, FAAD, Director, Phoenix Surgical Dermatology Group, Phoenix, AZ. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.
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Patterson JW. Disorders of epidermal maturation and keratinization. In: Patterson JW, ed. Weedon's Skin Pathology. 5th ed. Philadelphia, PA: Elsevier; 2021:chap 10.
Richard G. Ichthyoses, erythrokeratodermas, and related disorders. In: Bolognia JL, Schaffer JV, Cerroni L, eds. Dermatology. 5th ed. Philadelphia, PA: Elsevier; 2025:chap 57.