[4] Over the first few weeks the redness disappears and is replaced by dry, flaking skin on the arms, legs and around the belly button.

[5] After a few months hyperkeratosis develops with a dark grey or brown, ridged appearance on the ankles, knees and elbows.

The hyperkeratosis may grow more severe but more localized and is generally only present on flexural folds of the major joints.

[4] Ichthyosis bullosa of Siemens is an autosomal dominant genetic condition caused by a mutation in the gene for keratin 2e on chromosome 12.

The gene causing ichthyosis bullosa of Siemens is known and so a diagnosis can be confirmed by genetic testing.

[4] Treatments for ichthyosis bullosa of Siemens generally attempt to improve the appearance of the skin and the comfort of the patient.