Ichthyosis bullosa of Siemens is a rare skin disorder, a type of familial ichthyosis that is inherited in an autosomal dominant way. It is also known as bullous congenital ichthyosiform erythroderma of Siemens and as ichthyosis exfoliativa. It is a genetic disorder for which no cure is known, and it is estimated to affect about one person in 500,000. Ichthyosis is the general name for a family of conditions in which the skin is dry, thickened or scaly, and this form is distinguished from related ones by the name of the dermatologist Siemens attached to it.
Facts
Classification
ICD-10 ChapterXII: Diseases of the Skin and Subcutaneous Tissue 1 Communicable or Non-communicable Causative Agent
Causative Agent (category) First Described Sources
1. Ichthyosis bullosa of Siemens (Wikipedia)
Lead
a rare skin disorder
Lead [causative-agent-category]
It is a genetic disorder with no known cure
lead sentence 1
Ichthyosis bullosa of Siemens is a type of familial, autosomal dominant ichthyosis, a rare skin disorder.
Lead: disease category
Ichthyosis bullosa of Siemens is a type of familial, autosomal dominant ichthyosis, a rare skin disorder.
Article text
Ichthyosis bullosa of Siemens was first described by the German dermatologist Hermann Werner Siemens in 1937 from his study of an affected family.
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