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</div><div><span>Lamellar ichthyosis</span></div></div>
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<article><div id="d-article"><div class="page-info"><div class="page-title"><a name="start" id="start">
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</a><h1 class="with-also" itemprop="name">Lamellar ichthyosis</h1>
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</noscript></div><div class="main"><div id="ency_summary"><p>Lamellar ichthyosis (LI) is a rare skin condition. It appears at birth and continues throughout life.</p></div><section><div class="section"><div class="section-header"><div class="section-title"><h2>Causes</h2></div><div class="section-button"><button type="submit" aria-controls="section-1" role="button" title="Expand/Collapse section"><span class="icon icon-section-action">
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</div></div><div class="section-body" id="section-1"><p>LI is an <a test="test" href="./002052.htm">autosomal recessive disease</a>. 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.</p></div></div></section><section><div class="section"><div class="section-header"><div class="section-title"><h2>Symptoms</h2></div><div class="section-button"><button type="submit" aria-controls="section-2" role="button" title="Expand/Collapse section"><span class="icon icon-section-action">
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</div></div><div class="section-body" id="section-2"><p>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.</p><p>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:</p><ul><li>Difficulty in feeding</li><li>Loss of fluid (<a test="test" href="./000982.htm">dehydration</a>)</li><li>Loss of balance of minerals in the body (<a test="test" href="./002350.htm">electrolyte</a> imbalance)</li><li>Breathing problems</li><li>Body temperature that is not stable</li><li>Skin or body-wide infections </li></ul><p>Older children and adults with LI may have these symptoms:</p><ul><li>Giant scales that cover most of the body</li><li>Decreased ability to sweat, causing sensitivity to heat</li><li>Hair loss</li><li>Abnormal finger and toenails</li><li>Skin of the palms and soles is thickened </li></ul></div></div></section><section><div class="section"><div class="section-header"><div class="section-title"><h2>Treatment</h2></div><div class="section-button"><button type="submit" aria-controls="section-3" role="button" title="Expand/Collapse section"><span class="icon icon-section-action">
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</div></div><div class="section-body" id="section-3"><p>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.</p><p>Lifelong care of the skin involves keeping the skin moist to minimize the thickness of the scales. Measures include:</p><ul><li>Moisturizers applied to the skin</li><li>Medicines called retinoids that are taken by mouth in severe cases</li><li>High-humidity environment</li><li>Bathing to loosen scales</li></ul></div></div></section><section><div class="section"><div class="section-header"><div class="section-title"><h2>Possible Complications</h2></div><div class="section-button"><button type="submit" aria-controls="section-4" role="button" title="Expand/Collapse section"><span class="icon icon-section-action">
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</div></div><div class="section-body" id="section-4"><p>Babies are at risk for infection when they shed the collodion membrane.</p><p>Eye problems may occur later in life because the eyes cannot close completely.</p></div></div></section><section><div class="section"><div class="section-header"><div class="section-title"><h2>Alternative Names</h2></div><div class="section-button"><button type="submit" aria-controls="section-Alt" role="button" title="Expand/Collapse section"><span class="icon icon-section-action">
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</div></div><div class="section-body" id="section-Alt"><p>LI; Collodion baby - lamellar ichthyosis; Ichthyosis congenital; Autosomal recessive congenital ichthyosis - lamellar ichthyosis type</p></div></div></section><section><div class="section sec-mb"><div class="section-header"><div class="section-title"><h2>Images</h2></div><div class="section-button"><button type="submit" aria-controls="section-tnails" role="button" title="Expand/Collapse section"><span class="icon icon-section-action">
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<li><img src="//medlineplus.gov/ency/images/ency/tnails/1914t.jpg" alt="Ichthyosis, acquired - legs" title="Ichthyosis, acquired - legs" class="side-img"/><a href="../imagepages/1914.htm">Ichthyosis, acquired - legs</a></li>
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</ul></div></div></section><section><div class="section"><div class="section-header"><div class="section-title"><h2>References</h2></div><div class="section-button"><button type="submit" aria-controls="section-Ref" role="button" title="Expand/Collapse section"><span class="icon icon-section-action">
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</div></div><div class="section-body" id="section-Ref"><p>Martin KL. Disorders of keratinization. In: Kliegman RM, St. Geme JW, Blum NJ, Shah SS. Tasker RC, Wilson KM, eds. <em>Nelson Textbook of Pediatrics</em>. 21st ed. Philadelphia, PA: Elsevier; 2020:chap 677.</p><p>Moon M, Guerrero AM, Li Xiaoxiao, Koch E, Gehris RP. Dermatology. In: Zitelli BJ, McIntire SC, Nowalk AJ, Garrison J, eds. <em>Zitelli and Davis' Atlas of Pediatric Physical Diagnosis</em>. 8th ed. Philadelphia, PA: Elsevier; 2023:chap 8.</p><p>Patterson JW. Disorders of epidermal maturation and keratinization. In: Patterson JW, ed. <em>Weedon's Skin Pathology</em>. 5th ed. Philadelphia, PA: Elsevier; 2021:chap 10.</p><p>Richard G, Ringpfeil F. Ichthyoses, erythrokeratodermas, and related disorders. In: Bolognia JL, Schaffer JV, Cerroni L, eds. <em>Dermatology</em>. 4th ed. Philadelphia, PA: Elsevier; 2018:chap 57.</p></div></div></section>
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<section><div class="section"><div class="section-header"><div class="section-title"><h2>Review Date 11/18/2022</h2></div><div class="section-button"><button type="submit" aria-controls="section-version" role="button" title="Expand/Collapse section"><span class="icon icon-section-action">
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<div id="section-version" class="section-body"><p>Updated by: Elika Hoss, MD, Assistant Professor of Dermatology, Mayo Clinic, Scottsdale, AZ. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.</p>
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