LAMELLAR ICHTHYOSIS

Lamellar Ichthyosis (LI) is an uncommon inherited skin condition that manifests from birth. It is among the three inherited skin illnesses known as autosomal recessive congenital ichthyoses (ARCI), which are clinically significant. People’s body with lamellar ichthyosis produces skin cells at a regular pace. However, they do not split up from one another at the skin’s surface as they ought to. Furthermore, the body doesn’t remove the skin quickly enough, resulting in the formation of brown scales.

In addition, newborns born with lamellar ichthyosis are wrapped in a transparent membrane, giving the appearance that they are covered in plastic wrap. Their skin may seem split, tight, black, or red. These newborns’ skin might be so tight that it presses their lips outward. Also, the skin on their hands may be thick, making it difficult for them to stretch their fingers.

SYMPTOMS

Many kids with lamellar ichthyosis are conceived with a collodion membrane, a waxy, glossy, transparent coating of skin. As a result, these newborns are referred to as collodion babies. During the first two weeks of life, the membrane peels, and the skin behind the membrane becomes scaly and red, like a fish’s surface. A newborn with lamellar ichthyosis may suffer from various health issues:

  • Systemic or skin infections
  • Inconsistent body temperature
  • Breathing difficulties
  • Imbalance of electrolytes
  • Dehydration
  • Eating difficulties

These symptoms may be present in older children and adults with lamellar ichthyosis:

  • Thickened skin in soles and palms
  • Toenail and fingernail abnormalities
  • Loss of hair
  • Reduced sweating capacity, resulting in heat sensitivity
  • Massive scales that envelop the majority of the body

DIAGNOSIS

A physician could commonly establish a diagnosis by evaluating the damaged skin and the distinctive scales. Additional tests, like a skin biopsy, may be performed. This could be required to rule out other possible reasons for dry, scaly skin. Furthermore, persons might have to consult the following healthcare professionals to be diagnosed:

  • Ophthalmologist
  • Dermatologist
  • Clinical geneticist 

TREATMENT

Collodion newborns are frequently admitted to the newborn critical care unit (NICU). They are put in an incubator in high humid conditions. They will require additional feedings, and moisturizers must be used on the skin. Moreover, infants may generally go home when the collodion membrane has gone. Skincare for life entails keeping the skin hydrated to reduce the thickness of the scales. Among the measures are:

  • Bathing to remove scales
  • High-humidity conditions
  • In extreme situations, retinoids are administered orally
  • Moisturizers used on the skin

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