Ichthyosis

Inherited very dry, scaly skin. Lifelong, manageable, and the daily treatment burden is far heavier than most people realise.

What it is

A group of inherited conditions in which the skin does not shed normally, producing persistent dryness and scaling.

Ichthyosis vulgaris is the commonest and mildest, affecting perhaps 1 in 250 people, often with eczema and keratosis pilaris. X-linked ichthyosis affects males and causes darker, larger scales. Rarer severe forms present at birth and affect the whole body.

There is no cure, and the treatment is emollients and keratolytics, applied in large quantities, every day, for life.

**The burden of that treatment is the part that is under-appreciated.** People with moderate or severe ichthyosis spend an hour or more a day on skin care, use several kilograms of emollient a month, and need it prescribed in adequate quantity. Prescriptions of small tubes are a common and pointless obstacle.

Severe forms affect much more than skin: overheating because sweating is impaired, which is dangerous in hot weather and during exercise; recurrent infections; hearing loss from ear canal scale; eye problems from tight eyelid skin; and difficulty with fine hand movement from thickened palms.

Emollients on the skin and on bathroom surfaces are a serious fire risk. Fabric that has absorbed emollient burns rapidly, and there have been deaths. Anybody using them, particularly smokers and people using open fires, must know this.

Signs you might notice

Persistently dry, rough, scaly skin, usually from early childhood.

Scales that may be fine and white, or large, dark and plate-like.

Sparing of the skin creases in ichthyosis vulgaris, which is characteristic.

Very marked lines on the palms and soles.

Itching, cracking and painful fissures.

Overheating and reduced sweating in more severe forms.

In babies with severe forms: a tight shiny membrane at birth, feeding and temperature problems.

See a GP or dermatologist for: cracking and infection; overheating; hearing or eye problems; or inadequate quantities of emollient being prescribed.

Urgent: fever with spreading redness, pain and pus, which suggests infection in cracked skin; or overheating with confusion in hot weather. See heat exhaustion and heatstroke.

How it can affect day-to-day life

Emollient quantity is the practical battleground. Adults with widespread disease need 500 grams or more a week, and pump dispensers rather than tubes. Getting that prescribed reliably is often the single most useful advocacy anybody can do.

Bathing to soften and remove scale, then applying emollient to damp skin immediately, is the routine that works. Adding sodium bicarbonate or salt to the bath helps some people.

Urea, lactic acid and salicylic acid preparations break down scale and are more effective than plain emollients for thick scale, though they sting on cracked skin.

**Fire risk must be stated explicitly.** Clothing, bedding and bandages that have absorbed emollient ignite readily. No smoking, no naked flames, and bedding and clothing washed frequently and at high temperature.

Overheating needs planning: cool environments, water sprays, reduced exercise in heat, and awareness that the person cannot sweat normally.

Schools need to understand the condition, allow water and cooling, and treat the appearance questions from other children openly.

Psychological support matters; visible skin disease from childhood shapes a great deal.

Supporting someone well

Get emollients prescribed in adequate quantity, in pump dispensers, and use them several times a day.

Bathe to soften scale, then apply emollient to damp skin immediately afterwards.

Use urea or lactic acid preparations for thick scale, avoiding cracked areas.

**Know the fire risk.** No smoking, no open flames, and wash emollient-contaminated fabric frequently at high temperature.

Plan for heat: cooling, fluids, and reduced activity in hot weather.

Get ear canals checked for scale and hearing tested.

Get eyes checked where the eyelid skin is tight.

Ask for referral to a specialist ichthyosis service for severe forms, and for genetic counselling.

Ask for psychological support and for school to be briefed.

Where to get help

A GP practice for prescriptions and referral; dermatology, and a specialist centre for severe forms.

Ichthyosis Support Group are the UK charity for this condition.

British Association of Dermatologists publish patient leaflets on the different types.

Changing Faces support people with visible differences.