Ichthyosis – fish scale diseaseHereditary or acquired ichthyosis belongs to a group of skin diseases in which the keratinization of the epidermis is disturbed.
What is ichthyosis?
The name ichthyosis is derived from the Greek word for fish: ichthys. It describes the appearance of this scaly skin disease. The often rough, thickened corneal layer is reminiscent of fish skin due to the heavy scaling. The term ichthyosis is used to describe a group of hereditary skin diseases that have multiple inheritance patterns with varying degrees of severity.
The hereditary variant (ichthyosis vulgaris) is the predominant form of all ichthyosis diseases and occurs in about 1 in 100-250 people. X-linked recessive ichthyosis affects approximately 1 out of 4.000 people. Of the rarer forms, only 1 in 100 are affected.000-500.000 persons affected. Acquired ichthyosis is rare and usually due to an underlying systemic disease, excessive skin clearing, various forms of cancer or infection. Most forms of ichthyosis occur independently of sex.
Ichthyosis is a skin disease that is limited to the epidermis. Patients with ichthyosis usually have several gene alterations (mutations) that lead to skin barrier disorders. This results in increased fluid loss. A cornification of skin cells with the typical skin surface. In some cases, ichthyosis can already be detected at birth. Skin lesions range from mild redness and/or fine scaling to large, adherent, flaky scales. The most severe skin changes occur in the rarer hereditary forms of ichthyosis.
Sometimes there is an occurrence of several cases within one family. The severity and course of the disease is strongly dependent on the particular mutations.
Symptoms and symptoms
The dominant form of ichthyosis sets in at 2-6 months of age. The disease is characterized by scaling. Flaking can be seen. Many children with ichthyosis also suffer from hereditary hypersensitivity (atopy) in the form of asthma, eczema and hay fever. Ichthyosis leads to cosmetic problems. At times, cracking of the skin. Infections can develop in the skin cracks.
Even if the skin looks and feels normal at birth, it becomes increasingly rough and dry during infancy and toddlerhood. The scales are most clearly visible on the extensor sides of the arms and legs and are absent on the flexor sides. The dry skin can lead to itching. The resulting need to scratch leads to skin redness in the affected areas. Children with ichthyosis usually experience significant improvement during the summer months.
Ichthyosis can be recognized by symmetrical scaling of the skin, ranging from barely visible roughness and dryness to severe, keratinized flaking. Scales are small, irregular and polygonal. As they are often wavy at the edge, the skin has a rough surface. The color of the scales ranges from white to gray to brown. Darker skinned people usually have darker scales.
Most commonly affects the surfaces of the arms and especially the legs, and there is a marked difference between almost normal flexural folds and the surrounding dry, scaly skin. Dry soles of feet and palms are common and make the skin look dirty. Here the skin is very vulnerable, which can lead to painful cracks, especially in dry weather conditions. Infections can occur in these cracks.
The goal of treatment is to maintain skin hydration, prevent infection, and relieve itching. Moisturizers and bathing are the most important therapeutic measures. Depending on the form of ichthyosis, it is recommended to bathe once or twice a day. Usually bathing in pure water is enough, but you can also add some salt to the bath water if needed. Moisturize the skin daily and after each contact with water in the form of creams and ointments.
Ointments with urea loosen the callus, regenerate the skin barrier and have an antimicrobial and descaling effect. Glycerin-containing agents are also helpful. Less irritating to the skin. They can also be used in combination with urea. Treatment can be adapted to the type. Severity of the disease can be adjusted. Ichthyosis cannot be cured, but only treated symptomatically. However, therapy can lead to significant relief of symptoms. Improve the quality of life.
The most common form of ichthyosis, ichthyosis vulgaris, is not yet present at birth. Symptoms appear in most patients during the first few years of life. Often the degree of scaling increases until puberty, then decreases with age.
The prognosis in hereditary ichthyosis vulgaris is good. For many patients, the symptoms subside over the years. The prognosis for the rarer hereditary forms is highly dependent on the mutations involved, but the symptoms are usually more severe than in ichthyosis vulgaris. The prognosis for acquired ichthyosis depends on the severity of the underlying systemic disease.