Ichthyosis



Ichthyosis


Michael W. Peterson, DO

Cary Chisholm, MD

Clay J. Cockerell, MD





Key Facts


Terminology



  • Ichthyosis vulgaris (IV)


  • X-linked ichthyosis (XLI)


  • Lamellar ichthyosis (LI)


Clinical Issues



  • IV and XLI are related to prolonged retention of stratum corneum (retention hyperkeratoses)


  • LI is due to hyperproliferation of epidermis


  • Thick, dark scales on skin


  • Sites involved and prognosis varies with subtype


Microscopic Pathology



  • IV: Compact eosinophilic orthokeratosis



    • Decreased or absent granular layer


    • Decreased filaggrin production


  • XLI: Marked hyperkeratosis with normal granular cell layer


  • LI: Marked compact hyperkeratosis with normal granular layer







Lamellar ichthyosis presents as plate-like quadrilateral scale image over the extensor surface of the arm in this patient.






This example of LI shows very thick compact orthokeratosis image in the stratum corneum along with some mild acanthosis image.


TERMINOLOGY


Abbreviations



  • Numerous variants



    • Ichthyosis vulgaris (IV)


    • X-linked ichthyosis (XLI)


    • Lamellar ichthyosis (LI)


Synonyms



  • Greek word “ichthys” means fish


  • IV: Ichthyosis simplex


  • XLI: Steroid sulfatase deficiency


  • LI: Nonbullous congenital ichthyosiform erythroderma


Definitions



  • Group of heterogeneous disorders causing abnormal cornification (scale) of epidermis

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Jul 8, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Ichthyosis

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