Ichthyosis: a rare disease that affects the skin

Ichthyosis We should talk about ichthyosis because there are several forms of it of varying severity1,2. Ichthyosis is a rare genetic skin disease characterised by very dry skin and a fish-scale appearance. But what is ichthyosis? What are the symptoms? What are the causes? What are the different forms? What are the consequences of this condition? What can be done to reduce the symptoms?

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Summary

What is ichthyosis? What are the symptoms?

  • <p>Very dry and rough skin</p>
    Very dry and rough skin
  • <p>Significant flaking</p>
    Significant flaking
  • <p>Hyperkeratosis</p>
    Hyperkeratosis

The term ichthyosis comes from the Greek " ichthus " which means fish3, referring to the scale-like appearance of the skin of some patients. 
Ichthyosis is a dermatological condition characterised by:

  • Very dry and rough skin4
  • Significant shedding, i.e. removal of the surface layers of the skin in the form of small flakes5,6.
  • Hyperkeratosis, which is a thickening of the skin 

The severity of clinical signs varies: the upper body, arms and legs are the most affected areas7, but symptoms can affect the whole body5.
It is a chronic disease, meaning that the symptoms are permanent8

What causes ichthyosis?

What causes ichthyosis?

In the majority of cases, ichthyosis is a genetic disease and is, therefore, hereditary. It is caused by the mutation of one or more genes coding for the production of skin barrier components5. Hereditary ichthyosis appears at birth or in early childhood1.
However, there are acquired cases of ichthyosis that appear in adulthood. Their development may be linked to the presence of an underlying cancer (lymphoma, visceral cancer, etc.), malnutrition, or a skin condition such as systemic lupus erythematosus.

What are the different forms of ichthyosis?

There are two main types of ichthyosis: hereditary and acquired

Hereditary ichthyosis

Ichthyosis vulgaris:

Ichthyosis vulgaris:

The most common form (95% of people have this form). 
A baby is born with healthy skin and then, at around three months, patches appear on the soles of the feet and the palms of the hands. 
The symptoms gradually spread to the upper body (especially the back), and to the arms and legs where the skin becomes scaly.
These scales are quite thin and white. 
Ichthyosis vulgaris is caused by a mutation in a gene that codes for the synthesis of a protein involved in cell differentiation in the skin. 

X-linked ichthyosis:

X-linked ichthyosis:

The second most common form.
Affects about 1 in 5000 men9
It appears in the first few months of life and affects almost only boys. 
It is characterised by dark scales forming large flakes mainly on the arms and legs, with a dirty and unsightly appearance. Hyperkeratosis and scaling improve with age and summer sun. 
This is a more serious form than the previous one.

Photo source: DERMAWEB

Lamellar ichthyosis:

Lamellar ichthyosis:

This is much rarer and is characterised by large patches of thick, brown scales all over the body. They take on the appearance of laminated paper-like strips.

Harlequin's ichthyosis:

Harlequin's ichthyosis:

This is the most severe form of congenital ichthyosis but also the rarest (1 in 1 million children). 
A baby is born wrapped in a translucent, taut, shiny film of collodion covering the entire body.
This gradually gives way to large, very thick, ridged and hard scales all over the body.

Acquired ichthyosis

This usually appears in adulthood and can be generalised or localised. It is characterised by thin, dry and rough skin, scattered with flakes. It is not associated with itching.

What are the consequences of ichthyosis?

Patients suffering from ichthyosis tend to not sweat and are, therefore, more sensitive to external conditions (temperature, dryness, humidity) and have difficulty adapting to seasonal changes5.
Skin superinfections are more common and the lesions may have an unpleasant odour. 
Ichthyosis, when it affects the hands, can cause a decrease in the precision of certain gestures. In severe forms, it can often cause cracks on the soles of the feet, causing pain when walking.
 

What are the treatments for ichthyosis?

To date, there is no curative treatment for the various forms of ichthyosis. Management is mainly based on symptomatic and local treatment of skin dryness and hyperkeratosis.

  • <p>Hydrates the skin</p>
    Hydrates the skin
  • <p>Local treatments</p>
    Local treatments
  • <p>Oral treatment</p>
    Oral treatment

Keeping the skin well-hydrated10 : 

Emollients are the first line of treatment. Whatever the form of ichthyosis, there is an alteration of the skin's barrier function and an emollient must be applied daily. Moisturising the skin at least twice a day and after each bath or shower is recommended. The emollient must be suitable for sensitive or even atopic skin and combined with suitable hygiene products (without fragrance, with mild surfactants) so as not to irritate the skin. Appropriate daily hydration and hygiene will work in synergy to treat the symptoms of ichthyosis. In severe forms, medical care with emollients and full body wraps may be necessary.

Local treatment10:

These are mainly based on the use of keratolytic products, useful in case of thick and/or numerous scales. These are mainly urea-based products. They should be applied once or twice a day and then it is advisable to space out the applications in the maintenance phase.
Clinical trials are currently underway to evaluate the efficacy and tolerability of local treatments based on vitamin A derivatives.
 

Oral treatment10:

These are medicines with molecules called retinoids as their active ingredient. These are vitamin A derivatives11.
Other treatments, including injectable biotherapies prescribed for other dermatological conditions, are undergoing clinical trials for the treatment of ichthyosis.
 

DEXERYL: an ally of choice in the management of the various forms of ichthyosis

DEXERYL: an ally of choice in the management of the various forms of ichthyosis

As you can see, the basis for the treatment of ichthyosis is, above all, appropriate hygiene and daily moisturising of the skin. 
DEXERYL supports you with emollient skin care and cleansing products for the treatment of the signs and symptoms of dry skin, particularly in the case of ichthyosis. 

DEXERYL Emollient Cream

DEXERYL Emollient Cream

DEXERYL Emollient Cream can be used to protect the skin and treat the signs and symptoms of dry skin, particularly in the context of ichthyosis, thanks to: 

  • glycerol for its hydrating action
  • vaseline and paraffin for their protective role
Discover the Emollient Cream

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