A Few Things You Should Know About Ichthyosis!

Under normal circumstances, the body continuously renews its skin surface, production of new skin cells and allowing older cells to shed from the surface. Ichthyosis interrupts this balance either because too many replacement skin cells are products or because the skin cells do not separate well from the skin surface when it is their time to drop off. The result is an accumulation of skin cells into thick flakes that stick to the body and resembles with fish scales. Ichthyoses are a heterogeneous group of disorders characterized by generalized scaling like that of a fish (‘Ichthys’ means fish).
One out of every 250 people suffer from ichthyosis vulgaris, which is the mildest form of ichthyosis. Ichthyosis is another term for severe, relentless problems with dry skin that usually begin in childhood. It can be genetic or develop later in life.
Q. What is ichthyosis ?
Ichthyoses are a heterogeneous group of disorders characterized by generalized scaling like that of a fish (‘ichthys’ means fish).

Ichthyosis vulgaris                                                                                    Figure 1: Fish scales

Q. How does it occur?

Ichthyosis is mostly hereditary and starts soon after birth or in first year of life, but it can occur later in life because of some diseases like leprosy, lymphoma and due to some drugs like clofazamine.

Q. What are the common types of ichthyosis?

The major groups of inherited ichthyosis are ichthyosis vulgaris (IV), X-linked recessive ichthyosis (XLRI), lamellar ichthyosis (LI), epidermolytic ichthyosis (EI) and non-bullous congenital ichthyosiform erythroderma (NBCIE).

Q. What are the chances of having ichthyosis?

Ichthyosis vulgaris is by far the most common type of ichthyosis with an estimated prevalence of nearly  1 in 250 individuals other types of ichthyosis are much rarer and range from 1 in 10,,00 to 1 in 500,000.

Q. What is the common clinical presentation of ichthyosis?

Ichthyosis vulgaris presents about 3 months after birth. Dry skin and mild to moderate scaling appear after a few months. The scale of ichthyosis vulgaris is usually more prominent on the extensor surfaces of the extremities with flexural sparing. The diaper area tends to be spared. The scales are fine, white. On the lower legs, the scales are usually larger with an adherent center and detached, outward-turning edge. Scaling of the forehead and cheek can also be seen. In more severe disease, scaling extends to large areas of the trunk, scalp, forehead and cheeks and may be associated with itching. Clinical symptoms and severity depend on season and climate, improving during the summer and with increasing humidity, and worsening in a dry, cold environment. Although the ichthyosis is progressive during childhood, it usually improves with advancing age.

Ichthyosis vulgaris in skin

Q. How is ichthyosis diagnosed?

Ichthyosis is diagnosed based on history and typical clinical appearance, usually no other tests are required. Depending on type of ichthyosis and to confirm the diagnosis eye examination,  cholesterol sulfate level, skin biopsy  and genetic analysis may be done.

Q. Is ichthyosis communicable?

No, it is not an infectious disease and cannot be transmitted from one person to another by physical means.

Q. What are the chances of other sibling having ichthyosis, if one child has ichthyosis?

Ichthyosis is a hereditary disorder and chances of inheriting it depends on type of ichthyosis. In the most common ichthyosis vulgaris type chances of offspring having ichthyosis is 50% if one parent is affected, but the severity can vary a lot, sometimes it can very subtle. In X- linked recessive ichthyosis only males are affected.

Q. What are the problems which a child with ichthyosis likely to face?

Any child with ichthyosis is likely to have dryness of skin, which would increase in winter months and improve in summer months. Most of the children do not have any other problem, sometimes secondary infection can occur secondary to excoriations caused by itching. In case of x-linked recessive ichthyosis there may be non descent of testis for which a surgeon should be consulted.

Q. Is ichthyosis life threatening?

No, it is not life threatening. Though it looks cosmetically disfiguring but has very little impact on health.

Q. Is there a permanent cure for ichthyosis?

At present it is not possible to completely get rid of this disease because it is a genetic problem, but lots of research is underway and with gene therapy we may be able to cure this disease in future.

Q. What are the common treatment options available for ichthyosis?

Most important is to maintain adequate hydration of skin by using plenty of moisturizers. Applying them immediately after bath when skin is moist will help a lot and child will feel better. In case of thick scaling sometimes we may need to add keratolytic agents, but that decision should be left to dermatologists.

Children with ichthyosis should also be given calcium and vitamin D supplementation to prevent risk of rickets.

Q. Does steroids have any role in treatment of ichthyosis?

Usually no, steroids should rather be avoided because of their side effects

Q. What to do if I suspect that my child is having ichthyosis?

Fish like scaling from birth or during first year of life with exacerbation in winters suggests ichthyosis. You should sort a consultation with dermatologist to confirm the diagnosis. Best thing which can be done by knowing the correct diagnosis is avoidance of wrong medications given by quacks which can have adverse consequences on your child.