Ichthyosis Vulgaris

February 2, 2017

Ichthyosis vulgaris, also known as common ichthyosis or fish scale disease, is the most common form of ichthyosis. It is a skin condition, which is commonly inherited, and is characterized by accumulation of dead skin cells resulting in thick and dry scales on the skin. Its name is derived from the Greek word meaning “fish.

Ichthyosis vulgaris slows down the natural shedding of the skin cells. This results in accumulation of the keratin in the topmost layer of the skin. Due to the presence of the scales in ichthyosis vulgaris, it is sometimes called as fish skin disease or fish scale disease. The scales can be present at birth; however, they initially appear in early childhood. In some cases, ichthyosis vulgaris is not diagnosed at all, as people mistake them for extremely dry skin. There are very rare cases of severe ichthyosis vulgaris with the majority of them being mild. Sometimes ichthyosis vulgaris is associated with other skin conditions, such as eczema.

The disorder is not present at birth, but usually develops during the first year of life with an improvement often seen during summer and with age. The symmetrical scaling of the skin is most prominent on the extensor surfaces of the extremities and spares the flexural folds varying from barely visible roughness and dryness to marked scaling.

Causes

Ichthyosis vulgaris is the most common type of ichthyosis and is estimated to affect around 1 in 250 individuals.

Inheritence – Ichthyosis Vulgaris or Fish Skin Disease or Fish Scale Disease is an inherited condition occurring due to a genetic mutation, which is passed on by one or both the parents. Children who have inherited the defective gene from only one parent will have ichthyosis vulgaris which is milder; whereas children who have inherited defective genes from both the parents will have a severe type of ichthyosis vulgaris. Children who have inherited this condition commonly will have normal skin at the time of birth; however, later on, in the initial years of their life, their skin will develop roughness and scaling.

Genetic – If this condition is not inherited or is not the result of gene mutations, then it is known as acquired ichthyosis. This is a rare type and commonly starts during adulthood; and it also is associated with other medical conditions, such as thyroid disease, HIV/AIDS or cancer.

Risk Factors

Ichthyosis vulgaris is found in people of all races and of both sexes. Hereditary ichthyosis vulgaris is fairly common. As many as 1 in 250 children may have hereditary ichthyosis vulgaris. On the other hand, acquired ichthyosis vulgaris is rare and is found almost exclusively in adults.

Symptoms

The most common locations for ichthyosis vulgaris include –

Fronts (extensor surfaces) of the legs
Backs (extensor surfaces) of the arms
Scalp
Back
Forehead and cheeks, especially in younger children

The scales of ichthyosis vulgaris range in size from 1–10 mm and in color from white to gray to brown, with darker-skinned people often having darker scales. The legs are usually affected more than the arms. The creases on the palms of the hands and the soles of the feet are prominent and often crack during dry or cold weather. However, the scaling tends to improve in more humid or warmer weather.

The following areas tend to NOT be affected –

Face
Front of the neck
Abdomen
Folds in front of the elbows (flexural surfaces of the arms)
Folds behind the knees (flexural surfaces of the legs)

Hereditary ichthyosis and acquired ichthyosis look similar, and both are usually itchy. However, the acquired form occurs in people with many internal conditions, including –

Poor nutrition
Infections, such as leprosy or HIV/AIDS
Glandular diseases, such as thyroid or parathyroid problems
Sarcoidosis
Cancer, such as lymphoma or multiple myeloma
Use of certain medications, such as nicotinic acid, cimetidine, and clofazimine

Complications

Some people with ichthyosis may experience –

Overheating – In rare cases, the skin thickness and scales of ichthyosis can interfere with sweating. This can inhibit cooling. In some people, excess sweating (hyperhidrosis) can occur.
Secondary infection – Skin splitting and cracking may lead to infections.

Treatment

Medication

Alpha hydroxy acids (lactic acid, glycolic acid) in prescription ointments /creams help in controlling the scaling and increasing the moisture in the skin.
Retinoids are vitamin A-derived drugs and are given in severe Ichthyosis Vulgaris. They work by cutting down the skin cells production. Side effects include: Inflammation of the eyes and lips, hair loss and bone spurs. Retinoids are contraindicated in pregnancy, as they can cause birth defects.

To get the best results, use moisture-retaining creams or ointments after a bath or shower, so that moisture is maintained within the skin surface. Petrolatum, lanolin or urea-containing preparations are very helpful in maintaining skin moisture.

Alternative Treatment

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