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About ichthyosis

What is ichthyosis?

Ichthyosis (say ick-thee-oh-sis) is a group of genetic conditions that make the skin dry, thickened and scaly, usually all over the body and for life.

From the ISG’s condition factsheets · 6 minute read

Elliot, who has lamellar ichthyosis, in a studio portrait with one hand resting on his shoulder.

Faces of Ichthyosis: ElliotLives with lamellar ichthyosis

Key facts

28+subtypes, mainly inherited
From birthMost types are congenital: present at birth
ManageableNo cure yet, but symptoms can be treated

More than dry skin

Lots of people have dry skin conditions, such as eczema or psoriasis, but they tend to be patchy and come and go. In ichthyosis the scaling is continuous and usually affects the whole body.

Most types are congenital, meaning they are present at birth, and inherited, meaning they come from changes in genes, so they may run in families.

What causes the scaling?

Skin is constantly shedding and being replaced, and genes control how this happens. In ichthyosis, changes in those genes mean skin cells don’t work properly.

In some types, cells are made faster than needed and pile up. In others, they can’t detach, so they build up in layers.

Can it be treated?

There is no cure at present, but symptoms can be managed, mainly through:

  • regular, intensive moisturising, at least twice a day
  • sometimes antiseptics or antibiotics
  • occasionally retinoids, medicines that can reduce scaling
Treatment guides

Types of ichthyosis, A to Z

Find your type, or the type your child has been diagnosed with. Each links to our full factsheet.

A

  • Acquired ichthyosis

    Usually appears in adulthood. Often comes from natural dryness of the skin in later life, and rarely is linked to another condition or a medicine.

    Not inheritedAdults
  • ARCI

    Autosomal recessive congenital ichthyoses

    The family of conditions that includes lamellar ichthyosis, congenital ichthyosiform erythroderma (CIE) and harlequin ichthyosis.

    Group of types

B

  • Bullous ichthyosis

    Also called epidermolytic ichthyosis (EI), BIE or EHK

    Rare. From birth the skin is fragile, red and blisters easily. From early childhood it becomes thicker and scalier, and blistering eases.

    Present at birthRare

C

  • Collodion baby

    A description, not a diagnosis

    Describes how some babies look at birth: covered in what parents describe as a tight, shiny film, like a sausage skin. It is usually linked to a type of ichthyosis.

    NewbornsStart here if your baby was just born
  • Congenital ichthyosiform erythroderma (CIE)

    Also called non-bullous ichthyosis

    Very rare, about 1 in 300,000 births. Red skin with fine white scales. Most babies are born as collodion babies.

    Part of ARCIPresent at birth

E

  • Erythrokeratoderma variabilis (EKV)

    A very rare inherited skin disease, with around 50 affected families in the UK. Redness and thickened skin; 90% develop signs in their first year.

    Rare

H

  • Harlequin ichthyosis

    An extremely rare and severe inherited ichthyosis. Around five babies are born with it in the UK each year.

    Part of ARCIPresent at birth

I

  • Ichthyosis vulgaris

    New name: FLG-nEDD

    The most common inherited type. Ranges from mild dryness to thick scaling, and often runs in families alongside eczema, asthma or hay fever.

    Most commonStarts in childhood

L

  • Lamellar ichthyosis (LI)

    Very rare, about 1 in 200,000 births. Babies are usually born with a collodion membrane, then develop large, dark, plate-like scales.

    Part of ARCIPresent at birth

N

  • Netherton syndrome

    New name: SPINK5-sEDD

    A rare inherited condition. Very red skin with fine dry scales, often from birth or the first weeks. The skin barrier is “leaky”, losing heat and moisture.

    Present at birthClinical trials

P

  • Palmoplantar keratoderma (PPK)

    Thickened skin on the palms of the hands and soles of the feet. It often runs in families, and everyone in a family has the same type.

    Hands and feet

R

  • Rare ichthyoses and syndromes

    Including Sjögren-Larsson syndrome, Conradi-Hünermann syndrome, KID syndrome, Refsum disease and the trichothiodystrophies.

    Rare

X

  • X-linked ichthyosis (XLI)

    New name: STS-sEDD

    The second most common type, affecting about 1 in 6,000 boys and men. Brownish scales that are often worse in winter.

    Mainly malesInherited

Living with ichthyosis

The inherited forms tend to last for life, though symptoms can get milder over time. If you have a child with ichthyosis, you may need to help them deal with other people’s reactions. Starting school and the teenage years can be especially hard.

As an adult, starting college, university, a new job or a new relationship can bring difficult times too. The ISG has leaflets for younger children, teenagers and young adults, and for teachers and others who care for your child.