Definitions for ichthyosis bullosa of siemens

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  1. Ichthyosis bullosa of Siemens

    Ichthyosis bullosa of Siemens is a rare skin disorder which is a type of familial, autosomal dominant ichthyosis. It is also known as bullous congenital ichthyosiform erythroderma of Siemens or ichthyosis exfoliativa. It is a genetic disorder with no known cure which is estimated to affect about 1 in 500,000 people.

U.S. National Library of Medicine

  1. Ichthyosis Bullosa of Siemens

    An autosomal dominant form of ichthyosis characterized by generalized reddening of the skin (ERYTHEMA) and widespread blistering. The disease shows similar, but somewhat milder, clinical and histopathological findings to those in HYPERKERATOSIS, EPIDERMOLYTIC and is associated with the gene that encodes KERATIN-2A.

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