ichthyosis hystrix


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Related to ichthyosis hystrix: ichthyosis hystrix gravior

ichthyosis

 [ik″the-o´sis]
any in a group of skin disorders characterized by increased or aberrant keratinization, resulting in dryness, roughness, and scaliness of the skin. Many different metaphors such as alligator, collodion, crocodile, fish, and porcupine skin have been used to describe the various types and stages of ichthyosis. Most ichthyoses are genetically determined, but some may be acquired and develop in association with systemic diseases or may be a prominent feature in certain genetic syndromes. The term is commonly used alone to refer to ichthyosis vulgaris. (See Atlas 2, Part L.) adj., adj ichthyot´ic.
ichthyosis conge´nita (congenital ichthyosis) lamellar exfoliation of newborn.
harlequin ichthyosis the ichthyosis affecting a harlequin fetus.
ichthyosis hys´trix a rare form of epidermolytic hyperkeratosis marked by generalized, dark brown, linear, wartlike ridges somewhat like porcupine skin.
lamellar ichthyosis a congenital, chronic form of ichthyosis present at birth, inherited as an autosomal recessive trait, in which the affected infant is born encased in a collodionlike membrane (see collodion baby) that is soon shed, the skin then becoming covered with large, coarse scales with involvement of all of the flexures as well as the palms and soles. Universal erythroderma and pruritus are characteristic, and ectropion of variable degree is usually present. Formerly called congenital ichthyosiform erythroderma (nonbullous type). (See Atlas 2, Part K.)
lamellar ichthyosis of newborn lamellar exfoliation of newborn.
ichthyosis linea´ris circumflex´a a congenital autosomal recessive disorder present at birth, characterized by the presence of generalized redness and scaling of the skin associated with migratory lesions and hyperhidrosis of the palms and soles.
ichthyosis vulga´ris the most common form of ichthyosis, inherited as an autosomal dominant trait, having an onset sometime after the first year of life, especially near puberty. There is prominent fine scaling, principally on the extensor surfaces of the extremities and back (the flexures are spared and there is little scaling of the abdomen and face), together with accentuated markings and creases on the palms and soles; atopy is often present.
X-linked ichthyosis a chronic form of ichthyosis affecting only males, transmitted as an X-linked recessive trait, that may be present at birth or appear in early infancy. It is characterized by the presence of prominent, very adherent scales, often brown, especially on the neck, extremities, trunk, and buttocks.
Miller-Keane Encyclopedia and Dictionary of Medicine, Nursing, and Allied Health, Seventh Edition. © 2003 by Saunders, an imprint of Elsevier, Inc. All rights reserved.

bul·lous con·gen·i·tal ich·thy·o·si·form e·ryth·ro·der·ma

diffusely red, eroded skin at birth, with subsequent scaling, tending to improve in later life, characterized by generalized epidermolytic hyperkeratosis and autosomal dominant inheritance.
See also: epidermolytic hyperkeratosis.
Farlex Partner Medical Dictionary © Farlex 2012

ichthyosis hystrix

Linear nevus. The skin contains bands or lines of rough, thick, warty, hypertrophic papillary growths.
See also: ichthyosis
Medical Dictionary, © 2009 Farlex and Partners
References in periodicals archive ?
On the basis of the above constellation of symptoms (history, morphology of lesions, histopathological findings), a diagnosis of ichthyosis hystrix was made.
Ichthyosis hystrix is a descriptive name for a heterogeneous group of keratinization disorders sharing similar clinical features of massive, spiky or verrucous lesions clinically and epidermolytic hyperkeratosis histologically.4 It was first described between 1731-1851 in the Lambert family of Suffolk affecting 11 family members in four generations, the first one being Edward Lambert (along with his descendants,known as the porcupine men).
In 1902, Brocq5described ichthyosis hystrix as an atypical form of congenital bullous ichthyosiform erythroderma.

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