Ichthyosis vulgaris

Ichthyosis vulgaris: Description, Causes and Risk Factors:ICD-10: Q80.0Alternative Names: Hyperkeratosis congenita, ichthyosis simplex.Ichthyosis vulgarisIchthyosis vulgaris is considered one of the five main types of ichthyosis the others being Lamellar ichthyosis, congenital ichthyosiform erythroderma (CIE), X-linked Ichthyosis, and epidermolytic hyperkeratosis. Most varieties of ichthyosis affect only one person in several thousand or tens of thousands. It appears in approximately one person in every 250 to 300. The name is rarer than the disorder itself, which often goes undiagnosed because people who have it think that they have simple “dry skin” and never seek treatment.In ichthyosis vulgaris, the skin cells are produced at a normal rate, but they do not separate normally at the surface of the stratum corneum (the outermost layer of skin) and are not shed as quickly as they should be. The result is a build-up of scale. Only a portion of the body may be involved, but scaling is most common and most severe over the lower legs. The scale is usually fine and white. Scaling on the torso is less severe and the face is usually unaffected. If the face is affected, the scaling is usually limited to the forehead and cheeks. Often, the skin on the palms of the hands and the soles of the feet is thickened and may have exaggerated lines. Babies with ichthyosis vulgaris often appear normal when they are born, but the skin abnormalities will almost always begin to show up by their first birthday. Ichthyosis vulgaris sometimes improves in certain climates or during the summer. Ichthyosis vulgaris also intends to improve with age.Ichthyosis vulgaris was thought be caused by an autosomal dominant gene. If a person inherited the gene for ichthyosis vulgaris, the gene overshadowed the gene for normal skin and the person displayed the disease. However, researchers recently discovered that the disorder is semi-dominant. Profilaggrin (a protein that makes up most of the granules in the granular layer in the epidermis) was reduced or absent from the skin or people with vulgaris. Severely affected individuals have mutations in both copies of the profilaggrin gene, while people with only one copy of the mutated gene usually have a very mild skin problem.Symptoms:Severity of symptoms may vary widely among family members who have the condition. Symptoms include:Dry, scaly skin.
  • Tile-like, small scales, in polygon shapes.
  • Scales colored white, dirty gray or brown — with people with darker skin tending to have darker colored scales.
  • Flaky scalp.
  • Deep, painful cracks in your palms and soles in severe cases.
Diagnosis:A doctor can often make a diagnosis by examining your skin and the characteristic scales. If there's any doubt, he or she may perform other tests, such as a skin biopsy. This may be necessary to rule out other causes of dry, scaly skin.To diagnose ichthyosis vulgaris, your doctor also will take into account:Personal and family history of ichthyosis vulgaris.
  • Age when ichthyosis first started.
  • Presence of other skin disorders.
Treatment:There's no known cure for ichthyosis vulgaris, so the goal of treatment is to manage the condition.Alpha hydroxy acids, such as lactic acid and glycolic acid: Treatment can include prescription creams and ointments that contain acids that help control scaling and increase skin moisture.Retinoids: Your doctor may proscribe these vitamin A-derived medications in severe cases. They reduce the production of skin cells. Side effects from the medication may include eye and lip inflammation, bone spurs and hair loss, as well as birth defects if taken during pregnancy.Your doctor may also recommend heavy duty moisturizers. Creams and ointments work better than lotions. Apply these to moist skin immediately after bathing. You should use mild, non-drying soaps.NOTE: The above information is educational purpose. The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition.DISCLAIMER: This information should not substitute for seeking responsible, professional medical care.


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