Dermatology Terminology by Herbert B. Allen

Author:Herbert B. Allen [Allen, Herbert B.]
Language: eng
Format: azw3, epub
Publisher: Springer London
Published: 2010-01-19T05:00:00+00:00

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5.2 Ichthyosis Vulgaris

Ichthyosis vulgaris is the most common of a group of skin disorders characterized by dry, thickened, fish-like and plate-like scale. It can be either inherited or acquired. Inherited ichthyosis vulgaris is an autosomal dominant genetic disorder affecting the profilaggrin gene on chromosome 1q21. Defects in this gene result in an impaired skin barrier. It is also associated with atopic dermatitis, which is also associated with defects in profilaggrin maturation. Underlying causes of acquired ichthyosis include malignancy, lymphoma, HIV infection, hypothyroidism, use of certain drugs such as nicotinic acid, and sarcoidosis. Clinical features manifest during infancy or childhood for the inherited form and in adulthood for the acquired form. Disease severity may vary widely from localized disease with “plate-like scales” affecting the shins or forearms to widespread disease affecting almost all body surface areas; ordinarily, severity is decreased in the body folds. Complications arising from ichthyosis include overheating, decreased range of motion, and secondary infections. Treatment includes emollients, retinoids, α-hydroxy acids, lactic acids, salicylic acids, urea, propylene glycol, and topical steroids. In acquired ichthyosis, treatment of the underlying condition can also result in improvement.

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