Definition: 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.
Other names Siemens Ichthyosis Bullosa; Ichthyosis Bullous Type; Ichthyoses, Bullous Type; Bullous Type Ichthyosis; Bullous Type Ichthyoses; Ichthyosis, Bullous Type of Siemens; Ichthyosis, Bullous Type; Bullous Type of Ichthyosis
