Saturday, May 07, 2005


increasesed cohesiveness,abnormal keratinization, increased basal cell proliferation
abnormally thickened stratumcornerum, hyperkeratosis, thin nucleated epidermis
coarse, fish-like scales
Ichthyosis Vulgaris
Autosomal Dominant
- reduced or absent keratohyaline granules in epidermis
attenuated stratum granulosum - reduced profilaggrin
loose stratum corneum,basket-weave appearance
most common, begins earlychildhood, small white scales on extensor surfaces of extremities andon trunk and face, lifelong disease
abnormalities in lipid metabolism from drugs or other diseases
X- linked ichthyosis
deficiency of sterioid sulfatase
steroid sulfatase degrades cholesterol sulfate
persistent cohesion of stratumcorneum

Lamellar ichthyosis
autosomal recessive - disorderof cornification, defect in lamellar body secretion
increased cohesivenss of stratumcorneum, numerous keratinosomes, abnormally large amount ofintercellular substance
severe and generalized ichthyosis
Epidermolytic hyperkeratosis
autosomal dominant, mutation inkeratin genes in suprabasal epidermis
faulty assembly of keratintonofilaments, impaired insertion into desmosomes, prevents normaldevelopment of cytoskeleton -> epidermal lysis and vesicles
generalized erythroderma,blistering, ichthyosiform skin
Source: Rubin's Pathology 4th Edition

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