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KRT10 gene
Reviewed by Medicinsk Expert•Last updated: 2026-10-22•Gene

KRT10 gene

The KRT10 gene provides instructions for making keratin 10, a protein that forms structural networks in skin cells to protect against physical stress.

Normal Function

The KRT10 gene provides instructions for making a protein called keratin 10. This protein is produced in keratinocytes in the outer layer of the skin (the epidermis). Inside these cells, keratin 10 partners with keratin 1 to form keratin intermediate filaments. These filaments assemble into strong networks that provide strength and resiliency to the skin, protecting it from damage caused by friction and everyday physical stresses.

Health Conditions Related to Genetic Changes

Epidermolytic hyperkeratosis

Dozens of mutations in the KRT10 gene have been found in people with epidermolytic hyperkeratosis, a condition characterized by red, blistering skin at an early age and thick skin (hyperkeratosis) later in life. People with KRT10 mutations typically have NPS-type epidermolytic hyperkeratosis, which features thick skin on many parts of the body, excluding the palms of the hands and soles of the feet.

These mutations change a single amino acid in the keratin 10 protein, affecting how intermediate filaments interact to form networks. While the altered proteins still form filaments, the resulting networks are disorganized and do not function normally. This leaves skin cells fragile and prone to blistering in response to friction or mild trauma.

Ichthyosis with confetti

Also called congenital reticular ichthyosiform erythroderma, this disorder is characterized by red, scaly skin all over the body with small patches of normal skin that look like confetti. The mutations involved alter the genetic sequence, leading to an abnormal keratin 10 protein with an excess of the amino acid arginine. This abnormal sequence directs the protein into the cell nucleus, where it cannot form the necessary intermediate filament network.

In some cases, the mutated gene corrects itself through a process where genetic material is exchanged between chromosomes. This allows the cell to produce normal keratin 10, which remains in the cytoplasm, forming patches of normal skin that increase in number and size with age.

Other disorders

Cyclic ichthyosis with epidermolytic hyperkeratosis is another skin disorder caused by KRT10 mutations. It is similar to epidermolytic hyperkeratosis, but the skin changes disappear for short periods before recurring for weeks or months.

Other Names for This Gene

  • CK-10
  • CK10
  • cytokeratin 10
  • K10
  • K1C10_HUMAN
  • keratin 10, type I
  • keratin, type I cytoskeletal 10
  • keratin-10

Frequently Asked Questions

The severity often correlates with the specific mutation’s location in the KRT10 gene. Mutations affecting the highly conserved helix‑boundary regions tend to produce more pronounced hyperkeratosis. However, clinical expression can be unpredictable. Regular skin examinations, documentation of symptom progression, and, if available, functional studies of the identified variant can help estimate future severity.
bsolutely. KRT10‑related disorders are typically autosomal dominant, so first‑degree relatives have a 50% chance of carrying the same mutation. Genetic counseling can explain inheritance patterns, discuss predictive testing for asymptomatic relatives, and guide family planning options such as prenatal diagnosis or pre‑implantation genetic testing.
Yes, the “confetti” pattern of normal‑appearing islands within red, scaly skin is characteristic of ichthyosis with confetti, which is linked to somatic revertant mosaicism of KRT10 mutations. Diagnosis is confirmed by skin biopsy showing normal keratin 10 expression in the confetti patches and genetic testing of affected skin to identify the pathogenic KRT10 variant.
Use gentle, fragrance‑free cleansers and apply thick, occlusive moisturizers (e.g., petrolatum‑based) multiple times daily to keep the stratum corneum hydrated. For active blistering, apply non‑adhesive dressings and avoid friction by wearing soft, breathable fabrics. Keratolytic agents such as low‑dose salicylic acid or urea creams can reduce scaling, but start with low concentrations to avoid irritation. In some cases, oral retinoids (e.g., acitretin) are prescribed under specialist supervision to lessen hyperkeratosis.
Source: MedlinePlus, National Library of Medicine

MedlinePlus brings together authoritative health information from the National Library of Medicine (NLM), the National Institutes of Health (NIH), and other government agencies and health-related organizations.

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