Lamellar ichthyosis is an inherited skin condition, which is rather rare. In lamellar ichthyosis, the skin cells are manufactured normally, but they fail to divide normally at the surface of the skin’s outermost layer of skin and do not get shed as rapidly as they ought to. Consequently, there is a retention in the formation of scales.
Signs and symptoms of lamellar ichthyosis are:
- Lamellar ichthyosis is congenital. The babies are born covered with a clear membrane; they have skin that is usually red, tight and split.
- The membrane gets shed a few weeks after birth, and the baby is covered with dark, broad scales which are separated by grooves which may be superficial or deep.
- Those having lamellar ichthyosis have difficulty closing their eyes completely due to the tightness of the skin around the eyes. Occasionally, the skin around the eyes is so tight, that it causes the eyelids to turn outwards, thereby exposing the inner red lid and triggering irritation. This is called ectropion. Untreated, the cornea gets damaged and this affects vision.
- They have thickened nails, a reddened skin and thick skin on the palms of the hands and soles of the feet.
- Infants tend to develop infections often, get dehydrated and suffer from respiratory problems.
- Hair loss, abnormally formed fingernails and toenails, increased sensitivity to heat, and reduced sweating are other clinical manifestations.
- Some individuals have joint deformities.
Causes Of Lamellar Ichthyosis
Lamellar ichthyosis is a genetic condition caused by mutations which afflict several genes. 3 genes have been recognized; they are – 1 gene which encodes the epidermal enzyme Transglutaminase 1 and 2 genes which encode 2 enzymes 12(R) – lipoxygenase and lipoxygenase-3.
It is a recessively inherited disorder; in recessive disorders, the condition becomes apparent when one inherits a faulty gene from both parents. In case one receives one gene that is normal and one for the disorder, he will be a carrier and there won’t be any apparent features of the condition. The risk of a child developing the condition, from parents, both of whom are carriers, is about 25 % per pregnancy.
Treatment Options For Lamellar Ichthyosis
- Lamellar ichthyosis is managed by applying skin barrier repair formulas topically; these lotions contain ceramides, petrolatum or lanolin, and mild alpha-hydroxy acids.
- Severe cases are treated with oral retinoids. It helps decrease the scaling of the skin. Retinoids are used in severe cases only; given that, they cause bone toxicity and various other complications.
- There is no cure for the condition and lifelong management course is required.
- Conferring with an ophthalmologist is very important to manage all the eye related anomalies. Proper eye care for dry eyes is very crucial. Your health care provider will prescribe eye drops to keep the dry eye surface lubricated.
- Antibiotics are prescribed to manage secondary skin infections well.
- Lipid reducing medications are beneficial too.
- Moisturizing creams are necessary to battle dry surface of skin in the affected areas.
- Surgical skin grafts to repair large areas of severe dry skin are advocated.
- It is vital to maintain an optimal hydration status. Drink 3 litres of water daily.
- Avoid excessive exercising; moderate exercise is recommended, talk to your fitness instructor and develop a routine. Avoid outdoor activities during the hot weather and do not spend prolonged intervals of time in hot sunlight.