A Family Doctor's Tale - ICHTHYOSIS

DOC I HAVE ICHTHYOSIS

Ichthyosis presents with a scale like appearance of the skin like the scales of a fish.

Because of the scales there is reduction of sweat and oil secretion resulting in dry skin and dehydration.

Treatment is with petroleum jelly or oils which will help to hydrate the skin and eases the discomfort and itch of the patients.


Ichthyosis is a rare non contagious disease of the keratin of the skin where there is reduction of sweat and sebaceous secretions.

The word Ichthus means fish.

So Ichthyosis means fish like appearance of the skin.

People who are at risk of Ichthyosis have a family history of ichthyosis.

Most forms of ichthyosis are due to:

A.genetic conditions:


1.Ichthyosis vulgaris          95% -autosomal dominant
2.X-linked ichthyosis        -sex linked recessive
3.Ichthyosis lamellaris  - autosomal recessive usually in newborns
4.Harlequins Ichthyosis  -autosomal recessive
5.Bullous Ichthyosis         - autosomal dominant

B.Acquired Ichthyosis


6.Ichthyosis acquisita occur in :
leprosy,
hypothyroidism,
lymphoma,
sarcoidosis,
Hodgkin disease

7.Xeroderma - is a mild form of ichthyosis, occurs in middle aged and older patients.



Symptoms:

1.extensor surface of limbs covered with dry scales(rarely the whole body is covered).

2.axilla and antecubal fossa (elbows,armpits,groins,back of knee)not affected

3.Usually symptoms present in childhood but improve during puberty to becoming worst again in later life.

4.Rashes can be extremely itchy in xeroderma

5.The condition is worse in colder weather.

6.Hair may be thin and dry.

7.Nails are brittle and dry.

The diagnosis of Ichthyosis is made based on:


1.Appearance of the skin

2.Family history of Ichthyosis

3.Skin biopsy for microscopic examination.

The complications of Ichthyosis are:


1.keratisis with corneal and ocular surface disease

2.Ectropion of eyelids due to dehydration

3.Deafness in a Keratitis-Ichthyosis-Deafness (KID)Disease



There is no cure for Ichthyosis.

1.Symptomatic treatment with emolient creams such as petroleum jelly may help to hydrate the skin.

2.Itch may be controlled with antihistamines

3.Hydration with propylene glycol solution will help

4.Urea in emulsion creams has also help the dry skin

5.Topical or oral retinoic acid has brought some improvement to the skin.

6.Exposure to the sun may improve or worsen the condition.



It is a lifelong condition and need continuous treatment.



There is no known preventive measure for ichthyosis