Ichthyosis

When ichthyosis it comes to scaling and scaly skin to peel. The severity can range from mild, annoying skin dryness to severe, disfiguring disease. In addition, the ichthyosis often occurs in the context of systemic diseases. The diagnosis is made clinically. Emollients for the treatment and sometimes oral retinoids are used.

Ichthyosis different from just dry skin (xeroderma) by connecting with a systemic disease or drug use, heredity, severity, or a combination of these. Ichthyosis can be much more severe than xeroderma.

When ichthyosis it comes to scaling and scaly skin to peel. The severity can range from mild, annoying skin dryness to severe, disfiguring disease. In addition, the ichthyosis often occurs in the context of systemic diseases. The diagnosis is made clinically. Emollients for the treatment and sometimes oral retinoids are used. Ichthyosis different from just dry skin (xeroderma) by connecting with a systemic disease or drug use, heredity, severity, or a combination of these. Ichthyosis can be much more severe than xeroderma. Hereditary ichthyosis The classification of hereditary ichthyosis associated with the excessive accumulation of scale on the skin surface, will be based on clinical and genetic criteria (see Table: Clinical and genetic characteristics of some inherited ichthyosis). Some come with no additional features, and are not part of a syndrome (z. B. ichthyosis vulgaris, X-linked recessive ichthyosis, lamellar ichthyosis, congenital ichthyosiform erythroderma [epidermolytic hyperkeratosis]). Other ichthyosis occur as part of a syndrome that involves many organs. So are affected (hereditary mental retardation and spastic paralysis caused by a defect of fatty aldehyde dehydrogenase), which are autosomal recessive, skin and extracutaneous organs in Refsum’s disease and Sjögren-Larsson syndrome. For diagnosis and treatment, a dermatologist should be consulted and the purpose of genetic counseling, a doctor of Human Genetics. Severe lamellar ichthyosis in a child © Springer Science + Business Media var model = {thumbnailUrl: ‘/-/media/manual/professional/images/267_ichthyosis_slide_3_springer_high_de.jpg?la=de&thn=0&mw=350’ imageUrl: ‘/ – / media /manual/professional/images/267_ichthyosis_slide_3_springer_high_de.jpg?la=de&thn=0 ‘, title:’ Heavy lamellar ichthyosis in a child ‘, description:’ ‘credits’ © Springer Science + Business Media’, hideCredits: false, hideTitle : false, hideFigure: false, hideDescription: true}; var panel = $ (MManual.utils.getCurrentScript ()) Closest ( ‘image-element-panel.’). ko.applyBindings (model, panel.get (0)); Clinical and genetic characteristics of some inherited ichthyosis disorder inheritance patterns / prevalence outbreak type of dandruff distribution Associated clinical findings Ichthyosis vulgaris Autosomal dominant 1: 300 Childhood Fine Usually back and extensor surfaces, but not on intertriginous surfaces Normally many symptoms on the palms and soles atopy keratosis pilaris asthmaX-linked ichthyosis X-linked 1: 6000 (men) birth or infancy Tall, dark (may be fine) Prominent on the neck and torso Normal palms and soles corneal opacities cryptorchidism testicular cancer Lamellar ichthyosis (an autosomal recessive congenital ichthyosis) Autosomal recessive 1 : 300000 birth large, coarse, sometimes fine variable palm and soles changes majority of the body ectropium hypohidrosis with heat intolerance alopecia * Congenital ichthyosiform erythroderma (an autosomal recessive congenital ichthyosis) Autosomal dominant (mutations in about 50% of cases spontaneous) 1: 300,000 birth Dick, warty at birth: redness and blisters in adults include: scaling majority of the body especially warty in flexor folds bullae, frequent skin infections * This disorder is also known as bullous ichthyosiform erythroderma or epidermolytic hyperkeratosis. Acquired ichthyosis Ichthyosis can act as an early symptom of some systemic diseases (such. As leprosy, hypothyroidism, lymphoma, AIDS). Also, some drugs cause ichthyosis (z. B. nicotinic acid, triparanol, butyrophenone). In most cases the fine, dry scales are limited to the torso and legs. Sometimes but exist thicker scales affecting larger areas. The biopsy of the skin ichthyotic usually does not lead to diagnosis of systemic disease. However, there are exceptions, v. a. May occur in the fat on the legs shed sarcoidosis. Here show up in the biopsy usually the typical granulomas. Ichthyosis vulgaris on the abdomen © Springer Science + Business Media var model = {thumbnailUrl: ‘/-/media/manual/professional/images/270_vitiligo_slide_4_springer_high_de.jpg?la=de&thn=0&mw=350’ imageUrl: ‘/ – / media / manual /professional/images/270_vitiligo_slide_4_springer_high_de.jpg?la=de&thn=0 ‘, title:’ ichthyosis vulgaris at the abdomen ‘, description:’ ‘credits’ © Springer Science + Business Media’, hideCredits: false, hideTitle: false, hideFigure : false, hideDescription: true}; var panel = $ (MManual.utils.getCurrentScript ()) Closest ( ‘image-element-panel.’). ko.applyBindings (model, panel.get (0)); Treatment minimizing aggravating factors moistening and keratolytic Sometimes infection prophylaxis If ichthyosis is caused by a systemic disorder that underlying disorder must be treated so that ichthyosis is alleviated. Other treatments include emollients, keratolytics and avoiding dehydration. Moistening and keratolytic At each ichthyosis is impaired epidermal barrier function and moisturizers should be applied immediately after bathing. Substances that are applied to the skin, can have increased absorption. For example, hexachlorophene products should be avoided due to increased absorption and toxicity. should be 2 times a day an emollient – best pure petroleum jelly, mineral oil or lotions with urea or ?-hydroxy acids (such as lactic acid, glycolic acid and pyruvic acid) – are applied v. a. after bathing when the skin is still wet. By blotting with a towel is removed too much Brushed. Ichthyosis usually responds well to topical keratolytic propylene glycol. To shed to remove (eg. As when the ichthyosis is serious), the patient may be a preparation containing 40 to 60% propylene glycol in water, under occlusion (eg. As a thin plastic film or -tüte) every evening after hydration apply the skin (eg., by bathing or showering) and can act overnight. In children, the product should be applied two times a day without occlusion. Once the flakes are reduced, the application frequency can be reduced. Other useful topical agents are creams on ceramide base, 6% salicylic acid gel, hydrophilic petrolatum and water (in equal parts), and ?-hydroxy acids on different bases. Also topical Calcipotriolcreme has been used successfully, said vitamin D derivative in large areas and v. a. can cause hypercalcemia in young children. Retinoids are effective in the treatment of inherited ichthyosis. In most ichthyosis oral synthetic retinoids are effective. Acitretin (Systemic treatment) is effective in the treatment of most forms of inherited ichthyosis. When lamellar ichthyosis often 0.1% tretinoin cream or oral isotretinoin are effective. It should use the lowest effective dose should be used. Some patients develop under long-term (1 year) with oral isotretinoin bony exostosis, also more long-term side effects are possible. Tips and risks Oral retinoids are contraindicated in pregnancy because of its teratogenic effect. Acitretin should be avoided in women of childbearing age because of its teratogenicity and long duration of action. Prophylaxis of infection as long as thick intertriginous scales are present in patients with epidermolytic hyperkeratosis often a long-term treatment with 3 to 4 times daily 250 mg Cloxacillin (oral ingestion), or 3 to 4 times daily 250 mg erythromycin (oral administration) is required to to prevent secondary bacterial infections that cause painful, malodorous pustules. Through the regular use of chlorhexidine soap, the bacteria density can be reduced as well, but this also dries the skin out. Summary of ichthyosis can be purchased as an isolated disorder or as part of a syndrome or inherited. In patients with gradual onset ichthyosis an evaluation in terms of an underlying systemic disease must be carried out. Emollients, which accelerate molting (keratolytic) are effective in the treatment of ichthyosis.

Health Life Media Team

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