What Are Disorders of Cornification?
Disorders of cornification (also called disorders of keratinization or Mendelian disorders of cornification) is a broad group of skin conditions characterized by hyperkeratosis (a thickening of the skin), scaling and dryness, as well as varying degrees of inflammation and redness.
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Most disorders of cornification are inherited, though some develop in connection with an autoimmune or infectious disease, a nutritional deficiency or a malignancy. These disorders are caused by abnormal differentiation of keratinocytes, which are cells that produce the protein keratin. Keratin is the main component of the outermost layer of the skin, or the epidermis.
What Are the Types of Cornification Disorders?
Cornification disorders can be categorized as inherited and acquired ichthyoses, follicular keratoses, or palmoplantar keratodermas. For example, the inherited ichthyosis category comprises about 20 skin conditions, including:
- Ichthyosis vulgaris (the most common form of ichthyosis)
- Lamellar ichthyosis
- Epidermolytic hyperkeratosis
- Congenital ichthyosiform erythroderma
- X-linked ichthyosis
How Are Cornification Disorders Diagnosed?
Your child’s pediatric dermatologist will conduct a thorough examination of the affected skin and discuss his or her family history to make a diagnosis. Other tests, such as a skin biopsy, may be performed to rule out other causes of dry, scaly skin. Genetic testing may be appropriate to aid in diagnosis.
How Are Cornification Disorders Treated?
In general, the treatment goals for children afflicted with a cornification disorder are to:
- Preserve skin function
- Enhance quality of life by relieving itching and improving sleep and skin appearance
- Prevent complications, including infection, dehydration and poor skin growth
Depending on the type of cornification disorder your child has, his or her pediatric dermatologist will recommend such therapies as keratolytics, retinoids, emollients/moisturizers, antimicrobials, bath additives, medicinal shampoos, physical treatments (including hot and cold therapy) or targeted therapies.
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