Ichthyosis: Symptoms, Types and Treatment

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  • Topic: Ichthyosis, Genodermatoses, Epidermolytic hyperkeratosis
  • Pages : 4 (1387 words )
  • Download(s) : 125
  • Published : February 2, 2011
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While the ichthyoses constitute a rather large spectrum of about 20 cornification disorders that fit the definition and about 10 with more localized symptoms, they all are various permutations of a hyperkeratotic skin disorder that are characterized by an overgrowth and thickening of the corneal layer of the epidermis with localized erythema, and are classified from their; genetic or acquired origin, inheritance patterns, gene defects, etiologically as enzyme deficiencies, structural protein defects, regulatory protein defects, or other, and clinically as a syndromic ichthyosis, non-syndromic ichthyosis, or as a related condition. The term “ichthyosis” is derived from the Greek word ‘ichthys’ meaning ‘fish’. And aptly named as it is, a patient with ichthyosis will present with scaly dry ‘fish-like’ skin. The severity of scaling in ichthyotic patients runs a very broad spectrum, from severe to mild, where it blends into the realm of ‘dry skin’, or xerosis. The most minor of the ichthyoses, ichthyosis vulgaris, in its’ more mild forms, presents with light flaking and irritated apperence can be confused with simple xerosis. While the skin of patients with lamellar ichthyosis, is characterized by heavily pigmented, plate (armor) - like armor scales that are both obvious and often debilitating. And while all forms of ichthyosis present with a flaky or scaly appearance, several other characteristic clinical features are used as hallmarks of a particular types of ichthyosis so as to distinguish them from one another. In general congenital forms such as lamellar ichthyosis, congenital ichthyosiform erythroderma, epidermolytic hyperkeratosis, and ichthosis bullosa will present at birth with a yellow, tight and shiny, ‘collodion-like’ membrane which sheds in 2-3 weeks to reveal a flaky dry skin and in cases of ichthyosis bullosa, redness and blistering, especially over flexures. The traits of congenital ichthyosis, from obvious scaly appearance, to...
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