Ichthyosis Vulgaris

1 What is Ichthyosis Vulgaris?

Ichthyosis vulgaris is a disorder of cornification, characterised by persistently dry, thickened, “fish scale” skin. There are at least 20 varieties of ichthyosis, including inherited and acquired forms.

Ichthyosis Vulgaris has autosomal dominant inheritance, meaning an abnormal gene is inherited from a parent. Penetrance is 90%. Onset is delayed until at least 3 months of age.

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2 Symptoms

Symptoms of ichthyosis vulgaris vary in severity and tend to be less severe in a warm, humid climate.

  • Ichthyosis vulgaris is usually not present at birth. Most often it appears after about 2 months and in most cases before the age of 5. Symptoms may worsen up to puberty, and sometimes improve with age.
  • Dry scaly skin (xerosis) affects the extensor aspect of the limbs, scalp, central face and trunk. Skin folds are usually spared (neck, armpits, elbow and knee creases). There may be chapping (painful fissuring) of palms and soles.
  • Ichthyosis vulgaris is associated with keratosis pilaris (follicular hyperkeratosis) and hyperlinearity (pronounced skin lines) of palms and soles.
  • People with the mildest cases have no symptoms other than faint, tell-tale ""mosaic lines"" between the Achilles tendons and the calf muscles.

Atopic dermatitis (eczema) is present in 50% of people with ichthyosis vulgaris (and 8% of those with atopic eczema have the features of ichthyosis vulgaris).

When atopic dermatitis is associated with severe ichthyosis vulgaris, the dermatitis tends to be early in onset, severe, and more likely to persist into adult life. There is also an increased risk of asthma, rhinitis and food allergy.

3 Causes

Ichthyosis vulgaris is caused from loss-of-function mutations in the gene encoding the protein filaggrin (FLG), which is mapped on the epidermal differentiation complex on chromosome 1q21. The mutations lead to defective production of filaggrin.

Filaggrin is a filament-associated epidermal protein required for the binding of keratin fibres in epidermal cells, to form an effective skin barrier. It helps maintain the skin pH, retain moisture in the stratum corneum, and reduce trans-epidermal water loss (TEWL).

Xerosis or dryness results from the reduced skin hydration associated with defective filaggrin. Excessive scale results from the inability of the squames (skin cells) to remain hydrated as they move upward through the stratum corneum.

Hyperkeratosis results from compensatory repair mechanisms increasing cell proliferation.

4 Making a Diagnosis

Ichthyosis vulgaris is usually a clinical diagnosis. Mild ichthyosis is often just called “dry skin”.

Filaggrin mutations can be detected by research laboratories from a buccal smear or saliva sample.

On skin biopsy, histology shows hyperkeratosis in the stratum corneum and reduced or absent granular layer. Electron microscopy also shows reduced or absent keratohyaline granules, of which the main component is the protein pro-filaggrin, the precursor to the filaggrin.

5 Treatment

The main aims of ichthyosis vulgaris treatment are:

  • Reduce dryness,
  • Reduce scaling,
  • Reduce splitting and thickening of the skin. This is achieved with exfoliation and moisturizing on a regular, daily basis.

Treatments to keep the skin hydrated:

  • Apply emollients with high lipid content, such as lanolin cream (a sebum-like substance derived from wool-bearing animals).
  • Treatments to reduce scale
  • Bathe in salt water
  • Apply creams or lotions containing salicylic acid, glycolic acid, lactic acid or urea to exfoliate and moisturize skin. These may irritate active eczema.
  • Oral retinoid such as acitretin or isotretinoin can be prescribed in severe cases.
  • General tips
  • Apply lotions and creams to damp skin to trap in the moisture (within 3 minutes of showering/bathing).
  • Lotion and creams can be kept under occlusion for 1 or 2 hours with a cling-film wrap to enhance skin hydration.
  • Gently rub a pumice stone on wet skin to help remove thickened crusty skin.
  • Brush washed hair to remove scales from scalp.

6 Prevention

Several methods exist to prevent ichthyosis vulgaris, which include:

  • A nice soaking bath will help in softening the skin. After this, gently rub the skin with a rough-textured loofa or sponge to remove the thick scales.
  • Always pat your skin gently after a bath/shower, so that some moisture is left on the skin.
  • Always use mild soaps with added fats/ oils.
  • Do not use perfumes/scented and antibacterial soaps, as they worsen a dry skin.
  • After a bath, apply moisturizer/ lubricating cream, such as petroleum jelly, while the skin is still damp, this will help you seal in the moisture.
  • Using a humidifier will help in adding moisture to the air.

7 Alternative and Homeopathic Remedies

A few alternative and homeopathic remedies exist for ichthyosis vulgaris.

The most basic of all cures for dry skin includes drinking plenty of water. There are many homemade remedies for maintaining skin moisture, including the mixture of organic granulated sugar, honey and extra virgin olive oil. Another skin treatment mixture is made of eggs, honey, olive oil and rose water.

Natural oils – almond, avocado, bitter orange, lemon and lime – are good for treating dry skin. Crisco vegetable shortening can be used to treat dry skin.

Vitamins and mineral supplements can assist in curing dry skin:

  • Gotu Kola
  • Green tea
  • Honey
  • Hyaluronic Acid
  • Trypsin

A milk bath is also a common home remedy to moisturize skin.

Holistic herbs for curing dry skin include these:

  • Borage
  • Calendula flower
  • Chamomille tea
  • Coltsfoot
  • Comfrey plant
  • Dandelion tea
  • Geranium
  • Fennel
  • Hyssop
  • Lavendar oil
  • Oat extract
  • Patchouli
  • Peppermint tea
  • Rose
  • Sandlewood

8 Risks and Complications

There are several risks and complications associated with ichthyosis vulgaris.

Ichthyosis vulgaris may cause:

  • overheating: scales can reduce the capability to sweat, preventing an adequate thermoregulation
  • secondary infections: skin lesions favor cutaneous or more extended infections

Moreover it is often associated with atopic dermatitis and atopic asthma; in fact FLG deficiency provokes a paracellular barrier abnormality that reduces inflammatory thresholds to topical irritants, likely accounting for enhanced antigen penetration.